Spencer’s Pathology of the Lungassets.cambridge.org/97805215/09954/index/... · adenomatous rete testis hyperplasia 1473 adenosquamous carcinoma 1121–3, 1123 cell of origin 1121

Index

Note: page numbers in italicsrefer to figures and tables

AA amyloidosis 1337, 1338Abrikossoff’s tumor see granular

cell tumorsabscessblastomycosis 256Burkholderia cepacia 169Clostridium septicum 158Francisella tularensis 170hemolytic Streptococcus 164infection from

extra-pulmonarysources 151

Legionella micdadei 161Listeria monocytogenes 116Moraxella catarrhalis 171Nocardia asteroides 166Peptostreptococcus 159Staphylococcus aureus 163Yersinia pestis 175see also Wegener

granulomatosis,microabscesses

Acanthamoeba (amebiasis) 264,291, 294–5, 294

acetylcholine receptor (AChR)pathway 964

acid/alcohol-fast bacilli(AAFB) 207

acid b-galactosidase 423acid ceramidase deficiency

see Farber diseaseacid maltase deficiency

see Pompe diseaseacid sphingomyelinase 414, 416acinar dysplasia 82, 82Acinetobacter calcoaceticus

165, 166Acinetobacter pneumonia 148,

177acinic cell carcinoma 1138–41acinar cells 1139cell of origin 1138classification 1138clinical features 1138clinicopathologic correlation

1140

cytology 1140, 1140differential diagnosis 1140electron microscopy 1140genetics 1138glandular cells 1139histopathology 1138–9, 1139immunohistochemistry 1140intercalated ductal cells 1139macroscopic pathology 1138molecular findings 1138natural history 1140–1prognosis 1140–1radiographic features 1138typical carcinoid tumor

differential diagnosis 1167acinus 9, 10, 24–6, 81Actinomyces (actinomycosis)

pneumonia 177, 166acute capillary injury, antibody-

mediated rejection 781–2acute cellular rejection in lung

transplantation 769, 770,774–5, 779

airway inflammation withoutscarring 780–1

antibody-mediated rejectionassociation 782, 782

classification 778–81CMV pneumonitis distinction

777, 784diagnosis 775, 778–80, 781grades 774, 774–7, 776, 777,

778–81grading pitfalls 777–8morphological mimics 777,

777–8, 778, 779post-transplant

lymphoproliferativedisease – differentialdiagnosis 788–9

severe 777transbronchial biopsy 774

acute chest syndromesickle cell disease 680, 681

acute eosinophilic pneumonia(AEP) 577–80, 578

chronic eosinophilicpneumonia differentialdiagnosis 572, 580

clinical manifestations 577–8diagnostic criteria 577differential diagnosis 578–80diffuse alveolar damage 578–80histopathology 578, 580hyaline membranes 579laboratory findings 578natural history 580prognosis 580radiographic appearance 578smoking-related 579

acute febrile neutrophilicdermatosis 833

acute fibrinous organizingpneumonia (AFOP) 350,381–2, 382

dermatomyositis 827histological features 382polymyositis 827

acute interstitial pneumonia(AIP) 379–83, 381

clinical presentation 379differential diagnosis 382pathogenesis 382pathology 380–1, 381prognosis 383proliferative phase 380–1radiological findings 379–80treatment 383variants 381–2

acute lung injury (ALI) 342–58acute fibrinous organising

pneumonia 350acute phase 352airspace granulation tissue

polyps 347alveolar epithelial cells 355,

356alveolar epithelium damage

355alveolar re-epithelialization

356–7angiogenesis 356bronchoalveolar lavage 350clinical diagnosis 350–1coagulation system 355complications 344diffuse alveolar damage 342,

345–50

early disease 347edema fluid clearance 356electron microscopy 346endothelial cells 354–5, 355endothelial proliferation 356epidemiology 344etiology 342–4, 343exudative phase 345–6, 351–5fibroproliferative phase 355–7fibroblast migration 355fibroblastic focus 371fibrosis reversal 356hyaline membranes 345–6, 347incidence 344infection role 343, 350, 357inflammatory process

resolution 356intra-alveolar granulation

tissue removal 356macrophages 353, 354management 357–8mortality 344myofibroblast migration 355neutrophils 346, 351–3, 354non-pharmacological

interventions 358nonspecific interstitial

pneumonitis pattern 349open lung biopsy 351organizing 357, 450outcome 344pathogenesis 343–4, 351–7,

352, 357pathological features 345–50,

346, 348pathology 342pharmacological therapy

357–8platelets 354proliferative phase 346–9, 355–7radiographic features 344–5,

345repair process 187, 356–7resolution 357tracheal aspirates 350transbronchial biopsy 350–1

acute respiratory distresssyndrome (ARDS) 184,342–58

1565

www.cambridge.org© in this web service Cambridge University Press

Cambridge University Press978-0-521-50995-4 - Spencer’s Pathology of the Lung: Sixth EditionEdited by Philip Hasleton and Douglas B. FliederIndexMore information

http://www.cambridge.org/9780521509954

http://www.cambridge.org


acute silicoproteinosis (ASP)530–1, 531

acute vasculopathy, sickle celldisease 680

adenocarcinoma in situ (AIS)871, 889, 911, 1044, 1050

cytology 1058–9histopathology 1051–2morphological features 1065mucinous 1067–8, 1067,

1068non-mucinous 1052, 1053preinvasive lesion for lung

adenocarcinoma1049–50

prognosis 1066radiography 1065

adenoid cystic carcinoma1133–6

basaloid squamous cellcarcinoma differentialdiagnosis 1107–8

cell of origin 1133classification 1133clinical features 1133clinicopathologic correlation

1135cytology 1135, 1135differential diagnosis 1135electron microscopy 1135genetics 1133histopathology 1134, 1134immunohistochemistry 1135macroscopic pathology 1133,

1133metastases 1135, 1382, 1394molecular findings 1135natural history 1136prognosis 1136radiographic findings 1133typical carcinoid tumor

differential diagnosis1167

adenomatoid malformationssee congenitaladenomatoidmalformations

adenomatoid tumorof the peritoneum 1524of the pleura 1512, 1512, 1524

adenomatous rete testishyperplasia 1473

adenosquamous carcinoma1121–3, 1123

cell of origin 1121definition 1121differential diagnosis 1123genetics 1122–3histology 1121–2immunohistochemistry 1122incidence 1121mucoepidermoid carcinoma


prognosis/treatment 1123

adenovirus 193–4, 194lung transplantation 784–5, 784necrotizing hemorrhagic

pneumonitis 194pathology 193–4pneumonia 118, 182–3, 194smudge cells 194, 194ulcerative bronchiolitis 194

adiaspiromycosis 274, 275adrenal cortical carcinoma 1197,

1382adrenocorticotropic hormone

(ACTH) 972–3ectopic 973antiphospholipid syndrome

820coccidioidomycosis 262definition 342diagnosis 350diffuse alveolar damage 186–7,

350HSV infection 196malaria 298, 299radiographic features 345surfactant loss 26see also acute lung injury

aerodynamic equivalent diameter516

afferent autonomic nerves 12African–American people, lung

cancer 949agenon-small cell lung cancer

948tracheal stenosis 69

age-related EBV-associatedB-cell lymphoma 1344

aging of lungs 31–3, 624functional changes 32–3structural changes 31–2vascular remodeling 665–6

AIDS see HIV infectionair-crescent sign, angioinvasive–

disseminated aspergillosis235

air leaks 109–11air pollutionlung carcinoma 951–2see also environmental

pollutionairborne particle size 952airflow obstruction see airway

obstructionairspace development 1–4airspace enlargementdevelopment-related 623non-emphysematous 623starvation 623–4see also emphysema

airspace granulation tissuepolyps, ALI/ARDS 347

airway(s)anastomotic complications

in lung transplantation772–3

conducting 9development 1–4inflammation without scarring

in lung transplantation780–1

organization 7–8pneumonia 149–50wall thickness in asthma 567

airway-centered interstitialfibrosis 391, 464

airway disease, rheumatoidarthritis 811–13

airway obstructionclassification 607COPD 605, 607–8, 609, 612large airways 609small airways 609

airway surface liquid (ASL) 99,153

cystic fibrosis 99AL amyloidosis 1337, 1338Alaria 328alcohol ingestion, lung cancer

risk 958allergic bronchopulmonary

aspergillosis (ABPA)230–2, 598

bronchocentricgranulomatosis 576

Charcot–Leyden crystals 575diagnosis 232eosinophilic bronchiolitis

644histology 232middle lobe syndrome

633–4mucoid impaction of bronchi

575, 576pathology 574radiological appearance

232, 574allergic bronchopulmonary

fungal disease 573–5bronchiectasis 574–5bronchocentric

granulomatosis 575–6Charcot–Leyden crystals

574–5chronic eosinophilic

pneumonia differentialdiagnosis 571

clinical manifestations 573–4fungal hyphae 575‘gloved finger’ sign 574histopathology 574mucoid impaction of bronchi

574–5natural history 576–7organisms 573prognosis 576–7radiographic findings 574treatment 576

allergic granulomas, Churg–Strauss syndrome734, 735

allergic mucin 575allograft rejectionacute cellular rejection 769,

770, 774–5airway inflammationwithout scarring 780–1

antibody-mediated rejectionassociation 782

classification 778–81CMV pneumonitisdistinction 777, 784

diagnosis 778–80, 781grades 774, 774–7, 778–81grading pitfalls 777–8morphological mimics777, 777–8

post-transplantlymphoproliferativedisease differentialdiagnosis 788–9

antibody-mediated rejection768, 769, 781–3

bronchiolitis obliteranssyndrome 790–2

chronic vascular 792–3grading 774hyperacute rejection 771–2lung transplantation

hyperacute rejection 768altitude, hypoxic arteriopathy

686–8aluminum lung disease 549, 549alveolar adenoma 862–4, 862atypical adenomatous

hyperplasia differentialdiagnosis 917


864cytology 863, 863differential diagnosis 864electron microscopy 864genetics 862histopathology 862–3immunohistochemistry 863macroscopic pathology 862molecular findings 864natural history 864prognosis 864radiographic findings 862sclerosing hemangioma

differential diagnosis 872alveolar capillary barrier,

development 4alveolar capillary dysplasia

89–90, 89associated malformations 89genetic factors 90pulmonary vein misalignment

90alveolar damage, regional 382alveolar ducts 4, 24alveolar epithelial cells 24–6

Index

1566






acute lung injury 355,356, 348

hyperplasia 348, 916see also pneumocytes

alveolar damage, regional 382alveolar epithelium 909–10carcinogenesis 909–10damage in ALI 355reactive proliferation 915–16

alveolar hemorrhage 828bland 816, 817

alveolar lipoproteinosis, silicosis530–1

alveolar macrophages 27, 390iron-laden 52smokers 387viral infections 184–5

alveolar proteinosis, aluminumlung disease 549

alveolar rhabdomyosarcoma 123,1514–5, 1515

alveolar septal fibrosis 386alveolar soft-part sarcoma

1379alveolar volume 7alveolidevelopment 4, 5, 6–7postnatal 6

organization 9–10pores of Kohn 26, 26wall 25

amiloride-sensitive epithelialsodium channels(ENaC) 25

aminoaciduria 427–8aminorex, plexogenic arteriopathy

induction 673amiodarone toxicity 590, 590electron microscopy 590Niemann–Pick disease


nodules 591amniotic fluid, infection 115amebiasis 291–5, 292Balamuthia 291, 294–5free-living infections 294–5pleural infection 1425see also Acanthamoeba

(amebiasis), Entamoebahistolytica (amebiasis)

amosite cytotoxicity 1467amphiboles 512–13, 516, 1456,

1464asbestos bodies 517carcinogenic potential 1464fibers 516mesothelioma 1456

amphoterocin B 243, 244amyloid 1337calcifying fibrous tumor of the

pleura differentialdiagnosis 1430

deposition 814, 1326pleural 1423

senile pulmonary amyloid 32, 56tumor-associated 1340, 1340

amyloidosis 1340connective tissue disease 804diagnosis 1338immunohistochemistry 1339localized nodular 1338molecular pathology 1339nodular pulmonary 1331,

1339, 1339pleural fibrosis 1423pulmonary 1340, 1338rheumatoid disease 814, 1337,

1338Sjögren syndrome 1338, 1339systemic 1338, 1339tracheobronchial 1338transthyretin-derived 1337

anaerobic bacteria, pneumonia148, 155–75

Anaplasma phagocytophilumpneumonia 179

anaplastic large cell lymphoma(ALCL) 1342, 1356

anaplastic lymphoma kinase(ALK) gene, inflammatorymyofibroblastic tumor1277

anencephaly, ectopic glial tissue91

angioblasts 5angiocentric lymphoma 1335,

1337angiogenesis 4, 905–6, 925angiogenic squamous dysplasia

(ASD) 898angioimmunoblastic T-cell

lymphoma (AITL) 1346angiomatoid tumor of the

peritoneum 1524angiomyolipoma 1255–6, 1256angiomyolipoma 1249, 1250angiosarcomametastases 1383, 1392pleural 1518see also primary pulmonary

angiosarcomaAngiostrongylus

(angiostrongyliasis)313, 321, 321

angiotropic lymphomasee intravascular largeB-cell lymphoma

Anisakis (anisakiasis) 320ankylosing spondylitis 829,

829–30Ankylostoma duodenale 316Anopheles mosquito 297–8anthophyllite 1438anthracosis 217anthracotic pigmentation 536anthrax 177anti-basement membrane

antibodies, Goodpasturesyndrome 738, 739

antibody-mediated rejection(AMR) 768, 769, 781–3

acute cellular rejectionassociation 782

anti-HLA antibodies 782–3diagnostic criteria 782immunohistochemical criteria

782patterns of injury 781–2treatment 782–3

antigen detection,microbiological workup154–5

antigen presentation,mycobacterial infections213

antigen processing, modulationby mycobacterialinfections 212–13

antigenic drift/shift, influenzaA 187

antigenic stimulation, MALTlymphomas 1324

antineutrophil cytoplasmicantibodies (ANCA)

Churg–Strauss syndrome 732,735

diffuse alveolar hemorrhagein rheumatoid arthritis813

Goodpasture syndrome 739inflammatory bowel disease

834–5microscopic polyangiitis 731neutrophil interactions 711, 729pulmonary hemorrhage tests

711Wegener granulomatosis 713,

728, 834–5antineutrophil cytoplasmic

antibodies (ANCA)-associated diseases 711–40

drug-induced 713Goodpasture’s syndrome 382,

736–40microscopic polyangiitis 711,

729–31see also Churg–Strauss

syndrome, Wegenergranulomatosis

antioxidants, flavonoids 959antiphospholipid syndromeantibodies 819–20catastrophic 820, 820diffuse alveolar hemorrhage

816histologic features 820pulmonary hemorrhage

syndromes 741, 742pulmonary manifestations 820systemic lupus erythematosus

815, 819–20antiproteinase 3 622a-1-antitrypsin (A1AT)

deficiency 101, 606

antiproteinase 3 622diagnosis 620emphysema 619–20lung cancer risk 957panniculitis 622Wegener granulomatosis 714

a-1-antitrypsin (A1AT) molecule619

aortic sac 5APC gene mutations 865Apert syndrome, tracheal

stenosis 68apical cap 54, 1421–3, 1422, 1423differential diagnosis 1423epidemiology 1421fibrous 55histopathology 1422, 1422macroscopic pathology 1421–2pathogenesis 1422–3radiological findings 1421

aplasia of lung 74apoptosis 374, 903, 1465–6appetite suppressants 670, 673,

674argentiaffinity 1164arginine vasopressin (AVP) 972argyrophil nuclear organizer

regions (AgNOR) 1505Armillifer pentastomiasis 335,

334arsenic exposure, lung cancer

952–3arteriovenous malformations

(AVM) 1261–3clinical features 1261clinicopathologic correlation

1262differential diagnosis 1262hemangioma differential

diagnosis 1263hereditary hemorrhagic

telangiectasia 1261, 1262histopathology 1261macroscopic pathology 1261molecular findings 1261–2natural history 1262–3prognosis 1262–3pulmonary 1262radiographic findings 1261right-to-left shunting 1261treatment 1262–3

artefacts, biopsy 51–3, 51,52, 53

ASAH1 gene mutations 426asbestos 512–28atypical adenomatous

hyperplasia association921

background levels 513–16cell signaling 1464–5diffuse pleural fibrosis

association 1445environmental hazard 1460environmental sources 515,

1437–8

Index

1567






asbestos (cont.)exposure 513in buildings 1458lag period to benignasbestos-associatedpleural effusionoccurrence 1443

lag period to diffuse pleuralfibrosis 1446

lag period to pleural plaqueformation 1440

mesothelioma 1457–61fiber burden 522–4lung cancer 526

fiber deposition/clearance 516fiber potency and lung

carcinoma 525–6latent period from exposure to

mesotheliomadevelopment 1464

lung carcinoma with exposure525–8, 953, 957

diagnosis 526–7electron microscopy 526,527–8

risk 526smoking 525, 526tissue analysis 527–8

lung content in referencepopulations 516

mesothelioma risk 526non-occupational risk 513–16,

953occupational risk 512–13oncogenesis mechanisms 1464paraoccupational exposure 515peritoneal mesothelioma 1461permissible exposure level 516reference population levels

513–16risk in industries/occupations

515route of travel to pleura 1440smokinglung cancer 525, 526synergism 953

types 513uncoated fibers 525in whitewash 1459see also amphiboles, chrysotile,

crocidolite, erionite,mesothelioma, tremolite

asbestos-airways disease 520asbestos bodies 516–18, 517,

518, 522amphibole 517characteristics 517, 517chrysotile 517detection 518diagnostic value 520rounded atelectasis 1451sheet silicate 548

asbestos body concentration 515exposure standards 516lung fiber content 515–16

regulatory activity 516asbestos fiberscounts in diffuse pleural

fibrosis 1447mesothelioma 1456–7pleural plaques 1437route of entry into pleura 1440translocation 1408see also amphiboles, chrysotile,

zeolitesasbestosis 371–2, 518–25asbestos-related lung cancer

526benign asbestos-associated

pleural effusion 1436–7,1443–4

benign asbestos-related pleuraldisease 1436–54

pleural plaques 1423, 1430,1436–43

cigarette smoke exposure520, 525

classification 520clinical features 519definition 520, 522diagnosis 520differential diagnosis 519,

521–2diffuse pleural fibrosis 1436–7,

1444–8calcifying fibrous tumor ofthe pleura differentialdiagnosis 1430

epidemiology 518–19fibrosis 520, 522–5pleural 1446uncoated fibers 525

folded lung 1436–7, 1448–51grading 522–5, 523, 524histopathologic definitions 522histopathology 520, 523ischemic heart disease 1437lung carcinoma association

525, 526macroscopic pathology 520,

522mechanisms 519pathogenesis 519pleural thickening 1446prognosis 525radiological features 519–20,

519risk 513smoking association 520, 525,

1439–40see also mesothelioma

Ascaris lumbricoides (ascariasis)314–16

bolus 315clinical features 314–15diagnosis 316eosinophilic reaction 316epidemiology 314life cycle 315organism 314, 315

pathology 315–16strongyloidiasis differential

diagnosis 317treatment 316

Ascaris suum 316ascites, hepatic cirrhosis 1411Asherson’s syndrome 820, 820Askin’s tumor 1170, 1520aspergilloma 232–3, 233, 234calcium oxalate deposition 233clinical features 232conidiospore formation 233diagnosis 233pathology 232–3sarcoidosis 482zonation 233

Aspergillus infection(aspergillosis) 228–39

allergic forms 230–2, 231angioinvasive–disseminated

235–7clinical features 235diagnosis 237hematogenousdissemination 237

hyphae 237pathology 236–7

angioinvasive–disseminatedaspergillosis 235–7

antibody tests 238aspergilloma 232–3biomarker detection 238bronchocentric

granulomatosis 230, 231calcium oxalate crystals 234Candida differential diagnosis

267–8chronic cavitary 235chronic pulmonary 234–5cavitary 234, 235clinical features 234–5diagnosis 235fibrosing 234, 235necrotizing 234, 235nomenclature 234pathology 235

clinical manifestations 230conidia phagocytosis 230conidiophore 228culture 238cystic fibrosis 230, 233diagnosis 237–8differential diagnosis 238diffuse pneumonic 237echinocandin therapy 239epidemiology 229forms 230fungus ball 229Fusarium infection differential

diagnosis 244galactomannan detection 238genetics 229–30b-D-glucan detection 238granulomatous pulmonary 237host response 230

hyphae 229immunocompromised patients

229incidence 227–8invasive 236lung transplantation 229,

785, 786marijuana-induced 598molecular diagnostics 238mortality rate 239neutropenia 229organisms 228–9pathogenesis 230pathophysiology 230pattern recognition receptors

229–30pleural effusion 1412pleurisy 1413pneumonia 116, 226prognosis 239radiographic features 233rheumatoid nodules 807suppurative 237tracheobronchial 237, 237, 312treatment 238–9triazole therapy 238–9voriconazole 239see also allergic

bronchopulmonaryaspergillosis (ABPA)

aspiration 632, 633foreign body-type cells 222lipids 632pathological response 632route of injury in pneumonia

149aspiration bronchiolitis 466–7,

466aspiration pneumonia 466–7, 466,

632, 790dermatomyositis/polymyositis

827sarcoidosis differential

diagnosis 492aspiration pneumonitis 632asteroid bodies 55sarcoidosis 485, 485

asthma 563–8acute bronchiolitis 640airway epithelium 564–5airway lumen 564airway wall thickness 567aspergillosis 230basement membrane 565–6blood vessels 566Charcot–Leyden crystals 564,

567, 567clinical manifestations 563collagen ultrastructure

565–6Creola bodies 567, 567Curschmann’s spirals 567, 568cytology 567eosinophilic bronchiolitis 644eosinophils 566

Index

1568






epidemiology 563epithelial cell balls 567genetics 563goblet cell metaplasia 565histopathology 564–7inflammatory infiltrate 566laboratory findings 567lung cancer association 957lymphocytes 566macroscopic pathology 563–4mucus glands 567mucus plugs 563–4natural history 568neutrophils 566pathology 564, 565, 566plastic bronchitis 635prognosis 568pulmonary function tests 567radiological findings 563, 564T cells 566

atelectasis 51, 456biopsy artefact 51, 45see also rounded atelectasis

atopy, plastic bronchitis 635ATP-binding cassette A3

(ABCA3) gene mutations106

atrioventricular node cystictumor 1473

atypical adenomatoushyperplasia (AAH) 889,910–21

adenocarcinoma in situdifferential diagnosis1065

age factors 921alveolar adenoma differential

diagnosis 864angiogenesis 925asbestos exposure 921atypia 915carcinoembryonic antigen 926cell adhesion molecules 925–6cell cycle activity 922cell immortalization 925cell morphometry 922cellularity 915chromosome 3p loss 926chromosome 7 aneuploidy 926clinical relevance 927clonality 926–7concurrent lung

adenocarcinoma 921, 926cytofluorimetry 922cytology 915definition 912diagnosis 915differentialdiagnosis915–18,916estrogen receptors 925etiology 921extrathoracic malignancy

association 921gender association 921, 925genomic instability 926–7grading 915

ground-glass opacity 928histologic appearance

912–13, 913, 914, 914, 915hyperproliferation 922immunohistochemistry 914lesionslocation 912numbers 920, 920–1size 912, 915

lineage markers 925–6localized non-mucinous

bronchioloalveolarcarcinoma 922, 927, 928

macroscopic features 911–12,911, 912, 913

matrix metalloproteinases 926microscopic features 912–15molecular biology 921–2morphological features 1065multifocal disease 920multifocal micronodular

pneumocyte hyperplasiadifferential diagnosis 859

preinvasive lesion for lungadenocarcinoma 1049–50

prevalence 919–21, 920prognosis 927progression risk 927, 928, 929proliferation index 922radiographic features 911, 1065smoking association 921,

926–7subsequent invasion risk 927transcription factors 925tyrosine kinase signaling

pathway 923–5xenobiotic metabolizing

enzymes 926atypical mesothelial hyperplasia

(AMH) 1451, 1451–4,1452, 1508

diagnosis 1452–3differential diagnosis 1453survival 1453

autoantibodiesmixed connective tissue

disease 827scleroderma 820–1Sjögren syndrome 823, 825

autofluorescence bronchoscopy(AFB) 908

lung cancer 977specificity 909squamous dysplasia/

carcinoma-in-situ896–7, 908–9

autoimmune diseasepulmonary veno-occlusive

disease association 691sarcoidosis-like disease 490vasculitis 700see also named diseases

autoimmune pancreatitis,IgG4-related sclerosingdisease 1420

autopsy, specimen handling 45–7azygos lobe 8, 8Azzopardi phenomenon, small

cell lung carcinoma1162–3, 1163

B cell, immune response 154B-cell lymphomas 1324–43age-related EBV-associated

1344amyloidosis 1340Hodgkin’s lymphoma 1342,

1347immunohistochemical profiles

1329intravascular large B-cell

lymphoma 1336, 1337lymphomatoid granulomatosis

1331–6lymphoplasmacytic lymphoma

1331marginal zone lymphoma 825,

1324–9multiple myeloma 1340plasmacytoma 1342secondary pulmonary changes

1346see also diffuse large B-cell

lymphoma (DLBCL)B72.3 glycoprotein 1495Babesia (babesiosis) 300clinical features 300diagnosis 300epidemiology 300organisms 300pathology 300transmission 300vector 300

bacillary angiomatosis 177–8bacillary peliosis 177–8bacille Calmette–Guérin (BCG)

immunization 223Bacillus, pneumonia 177Bacillus anthracis 167, 177Bacillus cereus 168, 177Bacillus sphaericus 168bacterial infections 146–79barriers 153Gram-positive cocci 150hematogenous spread 152histologic features 155host defense 152–4inflammatory response 153–4innate immune response 154lung carcinoma 956–7lung transplantation 774management 154microbiological workup

154–5natural history 154necrotizing granuloma 150pathogenesis 152–4pneumonia 115–16, 146prognosis 154staining of bacteria 155

superinfection on viralinfections 185, 189–90

see also mycobacterialinfections, namedbacteria and diseases

Bacteroides fragilis pneumonia155, 158

bagpipe lung 75Balamuthia (amebiasis) 291,

294–5Balantidium coli 307baritosis 546barotrauma, cocaine abuse 597Bartonella pneumonia 177–8,

221basal cells 16–17hyperplasia 892–3, 892, 893

basal lamina see basementmembrane

basaloid carcinoma 1117–9cell of origin 1117cytology 1119differential diagnosis 1118histopathology 1117, 1118immunohistochemistry

1117–8, 1119pathology 1117prognosis 1118–9radiographic findings 1117

basement membrane 23, 27, 31bauxite mining 549Baylisascaris, toxocariasis

differential diagnosis 320Bcl-2 protein 904, 923, 1505Behçet syndrome 749–52,

831–2clinical manifestations 750, 750diagnostic criteria 749differential diagnosis 751–2epidemiology 749, 749exacerbations 832genetics 749–50heat-shock proteins 751hemoptysis 750histopathology 750–1infections 751macroscopic pathology 750natural history 752neutrophils 751pathogenesis 751pathology 750, 751, 831,

831–2, 832prognosis 752pulmonary artery aneurysms

831radiographic findings 750, 831remission 832

benign epithelial neoplasms847–79

alveolar adenoma 862–4bronchial papillomas 850–7multifocal micronodular

pneumocyte hyperplasia857–9

papillary adenoma 859–62

Index

1569






benign epithelial neoplasms(cont.)pulmonary endometriosis

875–9pulmonary hyalinizing

granuloma 872–4sclerosing hemangioma

864–72benign lymphoid proliferations

1317–23in interstitial lung disease

1317–8marginal zone lymphoma

differential diagnosis 1329pulmonary 1318

benign metastasizing leiomyoma(BML) 1244–7, 1245,1246, 1398


1247cytology 1247differential diagnosis 1247electron microscopy 1247genetics 1245histopathology 1245–7immunohistochemistry 1247lymphangioleiomyomatosis

differential diagnosis1254–5

macroscopic pathology 1245molecular findings 1247natural history 1247nodules 1245prognosis 1247racial factors 1245radiographic findings 1245treatment 1247

benzo[a]pyrene 950, 951Ber-EP4 glycoprotein 1495berylliosis 540, 541acute 540chronic 540, 541, 1424

berylliumexposure 540–1identification in lung

tissue 541lung cancer association 541sensitization 540toxicity 540

beryllium lymphocyteproliferation test 541

bevacizumab toxicity 592–3,1104

biglycan 621biopsyapplications 59–60artefacts 51–3, 62bronchial 59–60endobronchial 59–60inter-observer variability 62interface 49interpretation problems 62

large pleural 49misapplications 59–60open lung 59–60, 770problems 61–2surgical lung in interstitial

lung disease 366tissue problems 61–2transbronchial 59–61, 367transthoracic needle 59–60wedge 43–4see also video-assisted

thoracoscopic surgery(VATS)

Birbeck granules, Langerhans’cell histiocytosis 1348

bird-fancier’s lung 441, 446,467

Birt–Hogg–Dubé syndrome624–5, 1416

differential diagnosis 625genetics 624–5histology 625, 1418, 1419pathology 625, 625, 1418primary spontaneous

pneumothorax 1415radiographic appearance 625

bischloromethyl methyl ether(CMME) 953

black smoke measurement952

black spots, pleura 13, 31Blastomyces dermatitidis

(blastomycosis)228, 254–7

abscesses 198, 256antigen detection tests 256–7Candida differential diagnosis

267–8clinical features 254coccidioidomycosis differential

diagnosis 262cryptococcosis differential

diagnosis 262culture 256diagnosis 256–7differential diagnosis 257epidemiology 254histoplasmosis differential

diagnosis 250, 262immunocompromised

patients 257organism 254pathology 255–6, 255PCP differential diagnosis 272prognosis 257radiological appearance 254serological tests 256South American blastomycosis

differential diagnosis 264treatment 257see also Paracoccidioides

brasiliensis (SouthAmerican blastomycosis)

bleomycin toxicity 591–2Blesovsky syndrome 1448

blood cultures, microbiologicalworkup 154

blood group antigens 1496Bloom syndrome, lung cancer

risk 960blue bloaters, COPD 607blue bodies 56, 57blue clay 1459BMPR2 genehaploinsufficiency 670mutations 668–70, 674

body mass wasting, COPD 622–3bone marrow embolism 58, 58–9bone marrow transplantation,

amebiasis 294bone morphogenetic protein

4 (BMP-4) 5, 6bone morphogenetic protein

receptor 2 (BMPR2) 670pulmonary hypertension

668–70botryomycosis 163, 164, 165Neisseria mucosa 172pneumonia 176Staphylococcus aureus 164, 176with Streptococcus 165

brachial arch arteries 5BRAF gene mutation, lung

adenocarcinoma 1063brain natriuretic peptide (BNP),

pleural effusion incongestive heart failure1411

brain–thyroid–lung syndrome 1016Branhamella catarrhalis

see Moraxella catarrhalisbreast adenocarcinoma 1031breast carcinoma, metastases

1378, 1382, 1383,1389, 1395

breast mass, lymphangitic spread1385

bromocriptine, diffuse pleuralfibrosis induction 1445

bronchiabnormal origin/branching 73cartilage calcification 32compression 73congenital malformations 71–4development 1histology 15, 16mucus cell hyperplasia 19, 892mucus impaction 230–2allergic bronchopulmonaryfungal disease 574–5

organization 7, 8primary 8secondary 8segmental 9Sjögren syndrome 824–5submucosal elastosis 54tertiary 9

bronchi–biliary fistula 74bronchial arteries 30, 663anatomy 30, 665

angiography 98cystic fibrosis 98pathology 665post-obstructive vasculopathy

665therapeutic embolization 686

bronchial asthma, lungdevelopment 6

bronchial atresia 71–2, 72intralobar pulmonary

sequestration 83bronchial brushings, processing

41–2bronchial carcinogenesis

889–90animal models 890clonal patches 891etiology 891gene mutations 891loss of heterozygosity studies

907morphological changes 890tobacco smoking 891

bronchial circulatory system10–11

bronchial epithelium 15, 891bronchial gland collecting ducts,

oncocytic change 23bronchial inflammatory polyps

847–50cell of origin 847classification 847clinical features 848clinicopathologic correlation

849–50cytology 849differential diagnosis 850etiology 847histopathology 848–9macroscopic pathology 848natural history 850pathogenesis 847prognosis 850radiographic findings 848

bronchial isomerism syndromes73–4

bronchial lesions, pre-invasive 892bronchial papillomas 850–7glandular/glandular squamous

cell 855–7mixed squamous cell 855–7squamous cell papillomas

850–5bronchial pits, COPD 613bronchial probes 46, 47bronchial pseudoisomerism

68, 69bronchial smooth muscle 15bronchial stenosis 72bronchial submucosa 54bronchial submucosal glands,

oncocytic metaplasia 54bronchial veins 11, 663bronchial venous outflow 663bronchial venules 11

Index

1570






bronchial washings, processing41–2

bronchiectasis 627–32allergic bronchopulmonary

fungal disease 574–5aspiration 632aspiration pneumonia 632bronchial probes 47bronchiolitis obliterans 794broncholithiasis 634–5carcinoid tumor 632classification 628–9clinical features 629course 632cystic fibrosis 47, 103definition 627epidemiology 629etiology 628inflammation 629middle lobe syndrome 632–4,

634pathology 629, 630, 631pathophysiology 629plastic bronchitis 635post-tuberculous 223prognosis 632radiology 629, 629rheumatoid arthritis 813slab section 47tracheobronchopathia

osteochondroplastica635–6

treatment 632bronchiolar columnar cell

dysplasia (BCCD) 911bronchiole(s)development 1, 4diseases with/without adjacent

parenchymal disease643–8

histology 15, 17organization 7Sjögren syndrome 824–5terminal 9

bronchiolectasisfollicular 1318middle lobe syndrome 634pathology 631rheumatoid arthritis 813

bronchiolitis 636–48acute 638–40, 639histopathology 639–40

adenovirus 194chronic 804, 813, 813, 825classification 637–8clinical features 636–7constrictive 637, 639, 641,

641–3, 642, 643neuroendocrine hyperplasia641

toxic agent ingestion 642–3COPD 609–11definition 636diffuse panbronchiolitis 644eosinophilic 644

granulomatous 645–6, 647herpetic 186HTLV-1 644hypersensitivity pneumonitis

450, 455infections 151, 638–9, 640lymphocytic 780, 780, 781, 781measles 193mineral dust disease 646, 647parainfluenza viruses 192, 192pathogenesis 609–11pathology 386proliferative 640, 640radiological findings 637respiratory syncytial virus

191, 191rheumatoid arthritis 809smokers’ 644viral infections 185–6Wegener granulomatosis 718see also follicular bronchiolitis,

obliterative bronchiolitisbronchiolitis interstitial

pneumonia 391, 464bronchiolitis obliterans

see obliterativebronchiolitis

bronchiolitis obliteransorganizing pneumonia(BOOP) see cryptogenicorganizing pneumonia(COP)

bronchiolitis obliteranssyndrome (BOS)

differential diagnosis 792grading 792histopathology 792inflammatory component 792lung-resident mesenchymal

stromal cells 4lung transplantation 782–3,

790–2treatment 792

bronchiolization of alveolisee peribronchiolarmetaplasia

bronchitisbronchial pits 613chronic in coal workers’

pneumoconiosis 536chronic in COPD 612–14epidemiology 612pathology 612–14pathophysiology 612tracheobronchialseromucinous glandchanges 614

COPD 609–11, 612–14eosinophilic 644non-asthmatic eosinophilic 568clinical manifestations 568histopathology 568natural history 568prognosis 568

pathogenesis 609–11

plastic 635purulent in cystic fibrosis 100

bronchoalveolar carcinoma 1044see also adenocarcinoma

in situ (AIS)bronchoalveolar epithelium

909–10, 915–16carcinogenesis 909–10

bronchoalveolar lavage (BAL)41–2, 770

bronchoalveolar stem cells(BASC) 7

bronchocentric granulomatosis221–2, 577, 729

aspergillosis 230, 231, 575–6,576

bronchogenic carcinoma 531–2bronchogenic cysts 77–8, 78diagnosis 77etiology/location 77–8presentation 77

broncholithiasis 634–5, 635bronchomalacia 72–3associated anomalies 72–3management 73pathology 72

bronchoplastic resection 45–7bronchopneumonia 150, 774tuberculous 217, 217

bronchopulmonary chondroma1227

bronchopulmonary dysplasia106–9

chronic stage 108early exudative phase 107extremely low gestational age 6fibrotic cystic lung 106genetic factors 109hyaline membrane disease 106new 106–7, 108old 106–8pathogenesis 108–9pathology 107–8, 108prevalence 107prognosis 109pulmonary hypertension 108severe 107subacute fibroproliferative

stage 108surfactant absence 26thyroid transcription factor 1

1016Wilson-Mikity syndrome

differential diagnosis 109bronchopulmonary foregut

malformation 85bronchopulmonary segments

8–9, 9, 10bronchopulmonary sepsis,

chronic 223bronchoscopy 9bronchus-associated lymphoid

tissue (BALT) 15, 24,1316, 1317

acquired 1317

acute cellular rejectionmorphological mimic777, 778

hyperplasia 812inducible 1317lymphoid tissue 24smoker 15

Brucella, pneumonia 178Brucella melitensis 169Brugia malayi 308–9, 313brush cells 15, 22–3, 24distribution 22function 23microvilli 22, 22structure 22

bubble artefact, biopsy 52–3, 53Burkholderia cepacia 169, 178cystic fibrosis 99–100, 101, 178

Burkholderia pseudomalleipneumonia 169, 178

busulfan toxicity 591, 592

C-fibers 12C-mesenchymal–epithelial

transitional factor(C-MET) 1064, 1529

C-reactive protein (CRP) 1412cadherins see E-cadherin,

N-cadherincadmium pneumonitis 550calcified pleural granulomas

1430calcifying fibrous tumor of the

pleura 1429, 1429–30clinical features 1429differential diagnosis 1429–30pathology 1429prognosis 1430

calcitonin 1165calcium oxalate crystals 56, 486calretinin 1496–7, 1502–3Candida (candidiasis) 264–8antibody detection 267antigen detection 267ascending infection 115blood culture 267clinical features 265cystic fibrosis 265diagnosis 266–8differential diagnosis 267–8disseminated disease 265endobronchial spread 265epidemiology 264–5fungal autofluorescence 266germ tube production 267b-D-glucan detection 267hematogenous spread 265immune response 264immunocompromised patients

265incidence 227–8invasive 265latex agglutination test 267lung transplantation 785miliary 266

Index

1571






Candida (candidiasis) (cont.)molecular diagnostic methods

267organism 264pathogen recognition 264–5pathogenesis 264–5pathology 265–6pneumonia 115, 116, 264, 266prognosis 268radiological appearance 265sepsis/septic shock 265species complexes 264sputum culture 267systemic 265treatment 268

Candida albicans 264Candida dubliniensis 264Candida glabrata 264, 265Histoplasma capsulatum var.

capsulatum differentialdiagnosis 249

pathology 266PCP differential diagnosis 272

Candida krusei 264Candida parapsilosis 264, 265Candida tropicalis 264, 265cannabis abuse 597–8Capillaria (capillariasis) 320–1capillariesacute injury in antibody-

mediated rejection 781–2development 5lipid in 112

capillaritis 740–5clinical presentation 740connective tissue disease

pattern of involvement804

diagnosis 741diffuse alveolar hemorrhage 816laboratory studies 741radiographic findings 740–1systemic lupus erythematosus

742, 815Wegener’s granulomatosis 742

capillary network, development 5Caplan syndrome

see rheumatoidpneumoconiosis (RP)

carbon electrode makers 536carbon fibers 551–3carbon monoxide, lung

adenocarcinomaassociation 952

carbon nanotubes 553carborundum 544carcinoembryonic antigen (CEA)

926, 1495carcinogenesis 928–9alveolar epithelium 909–10peripheral lung

adenocarcinogenesismolecular biology 921–2

peripheral lung epithelium909–10

scar cancer theory 910see also bronchial

carcinogenesiscarcinogens 954, 955cigarette smoke 950, 951

carcinoid syndrome 1153carcinoid tumor 1155acinic cell carcinoma

differential diagnosis 1140atypical 1019, 1152, 1160clinical features 1153cytology 1160, 1160, 1161differential diagnosis 1167electron microscopy 1166histopathology 1160natural history 1169prognosis 1169typical carcinoid tumordifferential diagnosis1168

bronchiectasis 632classification 1151–2clear cell 1121, 1138cytology 1158, 1159, 1160dense core vesicles 1151diffuse idiopathic pulmonary

neuroendocrine cellhyperplasia 1176

fetal adenocarcinomadifferential diagnosis1074

fusiform cells 1157genetics 1166granular cell tumor differential

diagnosis 1242growth pattern 1157immunohistochemistry 1165macroscopic pathology 1155,

1155marginal zone lymphoma


melanin pigment 1158neuroendocrine markers 1027oncocytic 1158papillary/acinar architecture

1158plasmacytoma differential

diagnosis 1341primary pulmonary glomus

tumor differentialdiagnosis 1249

radiographic findings 1153–5,1155

sclerosing hemangiomadifferential diagnosis 872

small cell lung carcinomadifferential diagnosis1169

thyroid transcription factor 1expression 1018, 1019,1165

TNM classification 1012tumorlet differential diagnosis

1176–7, 1177

typical 1176atypical carcinoid tumordifferential diagnosis1168

clinical features 1152–3cytology 1159–60, 1166differential diagnosis 1167electron microscopy 1166histopathology 1157–60natural history 1169prognosis 1169variants 1157

carcinoid tumorlets see tumorletscarcinoma-in-situ (CIS)

see squamous dysplasia/carcinoma-in-situ(SD/CIS)

carcinomatous pulmonaryendarteritis 1389–90

carcinosarcoma 1197–205, 1201basaloid squamous cell

carcinoma 1198blastomatoid 1198, 1200–1,

1203–4, 1202, 1203clinical features 1202, 1203pathological features 1202,1203

pulmonary blastomadifferential diagnosis1203, 1214–5

clinical features 1198, 1199cytology 1203definition 1197–8differential diagnosis 1204electron microscopy 1204epidemiology 1200epithelial component 1198,

1203histopathology 1198–203, 1200immunohistochemistry 1203–4macroscopic pathology 1198metastases 1203molecular findings 1204natural history 1204–5pathological features 1199pleomorphic adenoma


primary pulmonarychondrosarcomadifferential diagnosis1238–9

primary pulmonary osteogenicsarcoma differentialdiagnosis 1236

prognosis 1204–5, 1204radiographic findings 1198,

1200sarcomatous elements

1201–3smoking 1198

cardiophrenic recess, blunting1412–3

Carney’s triad, pulmonarychondroma 1237

Carrington’s pneumonia 570–1caspases 1465Castleman disease 1268, 1322–3hyaline-vascular type 1322–3,

1323localized 1322–3mesothelioma differential

diagnosis 1514multicentric 1316, 1323clinical features 1323pathology 1323, 1324primary effusion lymphoma1355

plasma cell variant 1323pulmonary nodular lymphoid

hyperplasia differentialdiagnosis 1321–2

b-catenin gene mutation 1204,1212–3

cationic amino acid (CAA) 427,428

CD1a 1349, 1350CD44v6 906, 925–6CD57 1164CD205 see DEC205cdk inhibitors, mesothelioma

1468CDK4/cyclin D1 pathway

904–5cell adhesion molecules, atypical

adenomatous hyperplasia925–6

cell-associated sialomucins,mesothelium 1409

cell immortalization 906cellular immunity, giant cell

arteritis 746central nervous system (CNS),

paraneoplastic syndrome973–4

centrilobular fibrosis 467cerebral tissue, embolic

phenomena 90–1cervical cancer, metastases 1398cervical lymph nodes, metastases

1394cestodes 328–35cetuximab 593Chagas disease see Trypanosoma

cruzi (Chagas disease)Charcot–Leyden crystals 327allergic bronchopulmonary

aspergillosis 574–5, 575asthma 564, 567, 567chronic eosinophilic

pneumonia 569, 569treatment 328

chemodectoma see minutepulmonarymeningothelial-likenodules (MPMN)

chemokines 1317, 1412chemotherapyimmunosuppressive effects

1348

Index

1572






pulmonary veno-occlusivedisease 691

with radiation therapy on lungcarcinoma 594–5

childrenepituberculosis 218lung transplantation

indications 768mesothelioma 1460metastases 1376–7mucoepidermoid carcinoma

1127non-small cell lung cancer 948parainfluenza virus 192passive smoking 950pleuropulmonary blastoma

118–23, 1215pulmonary alveolar

proteinosis 395pulmonary

rhabdomyosarcoma 1242respiratory papillomatosis 850respiratory syncytial virus 191see also infants

Chlamydia trachomatis 159pneumonia 116, 175

Chlamydiaceae pneumonia 175Chlamydophila (Chlamydia)

pneumoniae 147, 175, 957Chlamydophila (Chlamydia)

psittaci 148, 175chloromethyl methyl ether

(CMME) 953cholesterol effusion 1429cholesterol granuloma 465, 465chondrosarcomametastatic 1380primary pulmonarychondrosarcomadifferential diagnosis1238–9

pulmonary chondromadifferential diagnosis 1237

myxoid 1521see also primary pulmonary

chondrosarcomachordoma, metastases 1387chorioamnionitis 115choriocarcinoma 1197metastases 1384, 1399

CHRNA3/5 alpha-nicotinicacetylcholine receptor957

CHRNA 3 gene 965Chromobacterium violaceum 170non-caseating granuloma 170pneumonia 178

chromogranin 21, 1026, 1027, 1164chromosomal aneusomy,

squamous dysplasia/carcinoma-in-situ 907

chromosome 3ploss in atypical adenomatous

hyperplasia 926smoking 907

chromosome 7 aneuploidy,atypical adenomatoushyperplasia 926

chromosome 15q, lung cancerassociation 964

chronic eosinophilic pneumonia(CEP) 568–73, 588

acute eosinophilic pneumoniadifferential diagnosis 580

associations 571causes 571Charcot–Leyden crystals 569,

569Churg–Strauss syndrome 569,

734, 736clinical manifestations 568desquamative interstitial


differential diagnosis 570–3eosinophils 569–70giant cells 569, 571histopathology 569–70, 569,

570, 571with lung cancer 572macrophages 569natural history 573prognosis 573radiographic findings 568–9,

569usual interstitial pneumonia

572chronic lung disease of

prematurity 107chronic lymphocytic leukemia/

small lymphocyticlymphoma (CLL/SLL)1347

chronic myeloproliferativedisorders, pulmonaryhypertension 684–6

chronic obstructive pulmonarydisease (COPD) 532–3,605–23

A1AT deficiency 606, 619–20,622

diagnosis 620acute bronchiolitis 640airway obstruction 605, 607–8,

609, 612large airways 609small airways 609

airway wall thickness 567basement membrane 612–13blue bloaters 607body mass wasting 622–3bronchial glands 613bronchial pits 613bronchiolar muscular

hypertrophy 610bronchitis/bronchiolitis 609–11chronic bronchitis 612–14epidemiology 612pathology 612–14

clinical manifestations 607–8

clinical phenotypes 607cor pulmonale 621–2course 611–12diaphragmatic dysfunction

623disorders 605emphysema 614–21A1AT deficiency 619–20classification 615–18genetics 607immunological process 621pathogenesis 620–1severity estimation 618–19smoking 620, 621

epidemiology 605–6, 606, 946gender 605–6genetics 606–7, 607hypoxic arteriopathy 688inflammation 611lung cancer risk 957lung transplantation 768macrophages 611Moraxella catarrhalis 147mucins 612mucus plugs 564, 610muscle dysfunction 623neutrophils 611osteoporosis 622panniculitis 622pathological changes 605, 609,

611, 613pathophysiology 609pink puffers 607prevalence 605–6prognosis 611–12pulmonary function tests 607–8pulmonary hypertension 621pulmonary vascular disease

621radiographic findings 608–9Reid index 614right ventricular dysfunction

621–2secondary pneumothorax

1415–6small vessel vasculitis 622smoking 609–11, 620, 621sporotrichosis 272susceptibility chromosomal

loci 957systemic effects 621–3systemic inflammatory

reaction 623tracheobronchial

seromucinous glandchanges 614

treatment 612chronic recurrent

thromboembolic disease(CRPE) 768

chronic thromboembolicpulmonary hypertension(CTEPH) 679–86

classification 679definition 679

epidemiology 679–80risk factors 679–80

chronic vascular rejection 792–3Chrysosporium parvum var.

crescenssee adiaspiromycosis

chrysotile 513asbestos bodies 517–18, 517cytotoxicity 1467environmental pollution 1437,

1438fibers 516mesothelioma 1456–7pleural plaque induction 1439

Churg–Strauss syndrome 731–6acute eosinophilic pneumonia

differential diagnosis 580airway involvement 733–4allergic granulomas 734, 735ANCA 732, 735asthma medication-induced 735autoantibodies 711chronic eosinophilic

pneumoniaassociation 569differential diagnosis 572

classification 731–2clinical manifestations 732–3,

733cytology 735definitions 732differential diagnosis 221,

310–11, 572, 734, 735–6,744–5

drug-induced 589eosinophils 734epidemiology 732filarial disease differential

diagnosis 310–11genetics 732histopathology 734, 734, 735humoral immunity 735IL-10 haplotype 732laboratory findings 735leukotriene receptor

antagonists 735macroscopic pathology 734,

734natural history 736pathogenesis 735peripheral neuropathies 733phases 732–3polyarteritis nodosa differential

diagnosis 744–5prognosis 736radiographic findings 733–4,

733, 734subgroups 732T-cell subpopulations 735tuberculosis differential

diagnosis 221vasculitis 734, 736

chylothorax 1428–9, 1428diffuse pulmonary

lymphangiomatosis 1259

Index

1573






chylothorax (cont.)lymphangioleiomyomatosis

1254, 1255cilia 18, 98abnormalities 96, 96

ciliated cells 15–16function 16structure 16

Clara cell secretory protein(CCSP) 7, 910, 1025

Clara cells see Club (Clara) cellsclear cell(s) see pulmonary

neuroendocrine cells(PNCs)

clear cell carcinoid 1121, 1138clear cell carcinoma 1120–1,

1121clear cell renal cell carcinoma 1258clear cell sarcoma 1258, 1489clear cell tumor of the lung

(CCTL) 1256–8, 1257acinic cell carcinoma


cell of origin 1256classification 1256clear cell carcinoma


clear cell squamous cellcarcinoma differentialdiagnosis 1106

clinical features 1256clinicopathologic correlation

1258cytology 1257–8differential diagnosis 872,

1029, 1106, 1121, 1138,1229, 1258, 1397

electron microscopy 1258epithelial–myoepithelial

carcinoma differentialdiagnosis 1138

histopathology 1257immunohistochemistry 1258macroscopic pathology 1256–7minute pulmonary

meningothelial-likenodule differentialdiagnosis 1229

molecular findings 1258natural history 1258prognosis 1258radiographic findings 1256renal cell carcinoma

metastases differentialdiagnosis 1397

sclerosing hemangiomadifferential diagnosis 872

clonal patches, bronchialcarcinogenesis 891

Clostridium, pneumonia 155–75Clostridium perfringens

pneumonia 155, 158Clostridium septicum 158

abscess 158pneumonia 155–75

Club (Clara) cells 7, 15, 17–18, 910electron microscopy 19function 17histology 18invasive lung adenocarcinoma

1055subsets 17–18

clubbing of fingers/toes 973mesothelioma 1469small cell lung carcinoma 1153solitary fibrous tumor of the

pleura 1435coagulation system, acute lung

injury 355coal bodies, ferruginized 536coal/coalmine dust 532–6cigarette smoking additive

effect 536dust reduction 533quartz content 533ranking 533

coal dust macule 533–4coal dust nodules 534coal workers’ pneumoconiosis

(CWP) 532–3, 534chronic bronchitis 536classification 533clinical features 533complicated 534differential diagnosis 535–6diffuse interstitial fibrosis 536emphysema 534, 536epidemiology 533lung carcinoma 536macular lesions 533–4nodular lesions 534pathogenesis 533pathology 533–4, 534, 535, 535progressive massive fibrosis

533, 534, 535–6radiological appearance 533

cobalt lung disease see hard metallung disease

cocaine abuse 596–7barotrauma 597pathogenesis 597pathology 596, 596toxicity 596

Coccidioides immitis(coccidioidomycosis)257–62, 258

antigen detection tests 262blastomycosis differential

diagnosis 257chronic pulmonary infection

259clinical features 259–61coccidioidoma 259–60complement fixation test 261–2culture 261diagnosis 261–2differential diagnosis 257, 262,

264

disseminated disease 261, 261epidemiology 257–9immunocompromised patients

260immunodiffusion tests 261latex particle agglutination

test 262miliary 261, 262necrotizing granulomas 259organism 257pathology 259–61primary pulmonary infection

259prognosis 262progressive pulmonary disease

260, 260radiological appearance 259serological tests 261–2skin tests 261South American blastomycosis

differential diagnosis 264treatment 262

coccidioidoma 259–60cold agglutinins, diffuse

panbronchiolitis 644colorectal adenocarcinoma,

metastases 1385, 1396,1396

colorectal carcinomametastatic 1031, 1032, 1396thyroid transcription factor 1

expression 1019common variable

immunodeficiency (CVID)lymphoma 1344primary effusion lymphoma

1355sarcoidosis-like disease 490

computed tomography (CT),micro-CT scanning 48

congenital adenomatoidmalformations 78–82, 79,1016

alveolar adenoma differentialdiagnosis 864

classification 78, 79–82cystic lesions 77, 79hemangioma differential

diagnosis 1263intralobar pulmonary

sequestration 83large cyst type 79–80, 79lung adenocarcinoma

association 1047malignancy risk 79management 78, 81–2mucigenic epithelium 80peripheral lung cyst 81presentation 78prevalence 78prognosis 81–2small cyst type 80, 80solid adenomatoid

malformation 80–1, 81subtypes 79–82, 81

congenital cystic lung 89congenital heart diseasewith hypo-/hyper-perfusion

693–6left-to-right shunts 696malformations with

pulmonary hyperflow696–7

plexogenic arteriopathy 673,697

pulmonary hypertension 667–8,696–7

pulmonary hypoperfusion 696congenital high airway

obstruction syndrome(CHAOS) 86

congenital lobar emphysema85–6, 85, 86

congenital lymphangiectasia87–8, 86

congenital malformations 66–95alveolar capillary dysplasia

89–90bronchus 71–4classification 76congenital lobar emphysema

85–6congenital pulmonary

lymphangiectasis 87–8diaphragmatic eventration

93–5diaphragmatic hernia 94–5enteric cysts 88–9incidence 66isomerism subtypes 74lung 74–95polyalveolar lobe 85pulmonary cystic disease

75–82pulmonary hyperplasia 86–7pulmonary hypoplasia 91–3pulmonary sequestration 82–4tracheal 66–71

congenital peribronchialmyofibroblastic tumor124–5, 125

congenital pulmonary airwaymalformation (CPAM),type 4 123

congenital pulmonarylymphangiectasis 87–8

diagnosis 87–8management 88

congestive heart failure, pleuraleffusion 1410–1

congestive vasculopathy 665, 692arterialization of veins 689–90associated conditions 689causative factors 689epidemiology 689histopathology 689–91medial hyperplasia 690pulmonary capillary

hemangiomatosisdifferential diagnosis 693

Index

1574






pulmonary hypertension 667,683–4, 689–91

pulmonary infarction 690–1pulmonary veno-occlusive

disease differentialdiagnosis 693

thrombotic arteriopathy 683–4vascular lesions 691

connective tissue 10, 31connective tissue disease 804–35acute exacerbation 804genetics 806idiopathic interstitial

pneumonia 806inherited 428–32interstitial lung disease

patterns 378lung cancer association 958lymphoid interstitial

pneumonia association463

nonspecific interstitialpneumonia pattern 378,379

patterns of pleuropulmonaryinvolvement 804, 805–6

pleural 1425–6pneumonia 371pulmonary hypertension 392,

697–8pulmonary veno-occlusive

disease association 691secondary complications 804undifferentiated 828–9, 829diagnostic criteria 829interstitial lung disease 829nonspecific interstitialpneumonia pattern 829

pathology 829see also named diseases and

conditionscor pulmonale, COPD 621–2cord funisitis 115coronary artery disease, pleural

plaque association 1436–7Coronavirus, SARS 182, 190corpora amylacea 56, 57corundum 549cotinine clearance 950–1coughHering Breuer reflex 1436solitary fibrous tumor of the

pleura 1435–6Coxiella burnetii pneumonia

148, 179craniosynostosis syndromes,

tracheal stenosis 68Creola bodies, asthma 567, 567CREST syndrome 697–8, 820,

822, 823cri du chat syndrome, lung

herniation 75crocidolite 1438, 1440, 1443,

1444, 1447, 1454, 1456,1459, 1459, 1467

Crohn’s disease 833–5, 834crospovidone 599, 600croup, parainfluenza virus 192Crouzon’s disease, tracheal

stenosis 68crush artefact, biopsy 52, 52crush phenomenon, small cell

lung carcinoma 1162–3,1167

Cryptococcus neoformans(cryptococcosis) 250–4

antigen detection tests 253–4Candida differential diagnosis

267–8clinical features 250–2coccidioidomycosis differential

diagnosis 262coin lesion 252culture 252–3diagnosis 252–4differential diagnosis 254, 262,

264, 267–8, 272epidemiology 250nodules 252organism 250pathology 251, 252PCP differential diagnosis 272pneumonia 253prognosis 254radiological presentation 252,

252serological tests 253South American blastomycosis

differential diagnosis 264tissue reaction 252treatment 254

cryptogenic fibrosing alveolitissee usual interstitialpneumonia (UIP)

cryptogenic organizing pneumonia(COP) 367, 463, 572

hypersensitivity pneumonitis455

lymphoid proliferation 1318Cryptosporidium

(cryptosporidiosis) 305–7bronchial 307clinical features 306diagnosis 307epidemiology 305–6fecal detection 306organism 306pathology 306schizogony 234treatment 307

Cryptosporidium hominis 306Cryptosporidium parvum 306Cryptostroma corticale spores 453crystal-storing histiocytosis

412, 413Curschmann’s spirals, asthma

567, 568Cushing syndrome 971, 972–3carcinoid tumor 1153small cell lung carcinoma 1153

CXCR3 chemokine receptor 28CXCR4 chemokine receptor

28, 1235cyclin D1 904, 909, 923cyclins, mesothelioma 1468cyclo-oxygenase 2 (COX-2) 906Cyclospora cayetanensis 307CYP1A1 see cytochrome P450

1A1cystic echinococcosis 328–34cystic fibrosis (CF) 97–101airway pathophysiology 99airway pus 99airway surface liquid

movement disruption 99aspergillosis 230, 233bronchial arteries 98bronchial wall 102bronchiectasis 47, 103Burkholderia cepacia 99–100,

101, 178Candida infection 265cartilage destruction 103changes in care 97clinical presentation 97–8clinical signs 98defect 99diagnosis 97genetics 98hilar lymph node 103histology/histopathogenesis

100–1infections 99–100, 178inflammation 99–100lung development 6lung disease progression 97–8mucosal gland hyperplasia 102pediatric lung transplantation

768pneumothorax differential

diagnosis 1419Pseudomonas aeruginosa

infection 99, 100pulmonary hypertension 99, 103purulent bronchitis 100secondary pneumothorax

1415–6serous gland hyperplasia 102sputum 97submucosal glands 99

cystic fibrosis transmembranereceptor (CFTR) 18, 98

cystic pleuro-pulmonaryblastoma (CPPB) 79

cysticercosis 333, 333–4cytochrome P450 1A1lung cancer risk 960pleomorphic carcinoma 1191–2polymorphisms 891, 949

cytochrome P450 enzymes 949cigarette smoke metabolism

950, 951cytokeratin(s)immunohistochemistry 1027mesothelioma 1498

biphasic 1502lung adenocarcinomadistinction 1496

neuroendocrine tumors 1165preinvasive lesions 906–7sarcomatoid mesothelioma

1502cytokeratin 5/6lung adenocarcinoma

immunohistochemistry1061

mesothelioma diagnosis 1498sarcomatoid mesothelioma

1502squamous cell carcinoma


cytokeratin 7, 1020, 1028cytokeratin 20, 1028cytokinesBALT 1317Langerhans’ cell histiocytosis

1351cytology specimen processing

41–2cytomegalovirus (CMV) 116–17,

194–5alveolar type II cells 195antigen 196chronic lung disease in infant

117clinical signs 195Cowdry type B inclusions 195cytoplasmic inclusions 195,

196, 784diagnosis 195, 783disease 783HIV infection 195, 196immunosuppression 195infection 783–4inflammatory response 784tissue invasive disease 783viral syndrome 783

cytomegalovirus (CMV)pneumonitis 184, 783

acute cellular rejectionmorphological mimic777, 784

lung transplantation 777,783–4, 784

miliary infection 195organ transplantation patients

195cytotoxic drugs, lymphoma

association 1345

D2-40, mesothelioma diagnosis1498–9, 1509

DEC205 (CD205) 28decorin 621b-defensin 2 (b-D2) 18–19defensins, viral infections 184delayed-type hypersensitivity

reactions, mycobacterialinfections 213, 217

Index

1575






dendritic cell(s) 27–8histoplasmosis infection 245myeloid 27–8plasmacytoid 27–8

dendritic cell sarcoma, follicular1352, 1352

dense core vesicles (DCV) 21, 1151carcinoid tumor/small cell

lung carcinoma 1151dental technician’s

pneumoconiosis 545, 545,546, 547

dermatofibrosarcomaprotruberans, metastases1392, 1400–1

dermatomyositis 825–7acute fibrinous organizing

pneumonia 827amyopathic 825anti-Jo-1 826aspiration pneumonia 827diffuse alveolar damage 826interstitial lung disease 826lung carcinoma 827, 958nonspecific interstitial

pneumonia pattern 826organizing pneumonia pattern

826, 826paraneoplastic syndrome

974–5pathology 825prognosis 826usual interstitial pneumonia

pattern 826desmin 1453, 1505desmocollin-3 1029desmoid tumor 1521extra-abdominal and solitary

fibrous tumor of thepleura differentialdiagnosis 1435

primary pleuropulmonary 1520desmoplastic small round cell

tumor 1171, 1518, 1519,1171

desmosomes, squamous cellcarcinoma 1103

desquamative interstitialpneumonia (DIP)367, 385, 388–91

chronic eosinophilicpneumonia differentialdiagnosis 573

clinical presentation 388differential diagnosis 389–90Gaucher disease differential

diagnosis 412histopathology 388–9, 390natural history 390pathogenesis 390prognosis 390radiological findings 388, 388

desquamative interstitialpneumonia-likereaction 388

desquamative interstitialpneumonitis 114

development of lung 1–7airspace 1–4airway 1–4alveolar stage 3, 4, 5canalicular stage 4genetic factors 5–6postnatal 6pseudoglandular stage 2, 1–4radial count estimation 4regulation 5–6saccular stage 4sex effect on growth 6stages 3vascular 4–5vascular remodeling 665–6

developmental delay 6diaphragmagenesis/aplasia 95dysfunction in COPD 623eventration 93–5, 94

diaphragmatic hernia 94–5, 95pathology 95presentation 94–5prognosis 95pulmonary hypoplasia 98tracheal stenosis 68

diaphragmatic plaque 1441DICER genemutations 122–3upregulation in localized

non-mucinousbronchioloalveolarcarcinoma 927

dietgenetic makeup 958lung cancer 958–60

dietary restriction, lung cancer 960diffuse alveolar damage (DAD)

379–83acute eosinophilic pneumonia

578–80acute exacerbation of

idiopathic pulmonaryfibrosis 384

acute lung injury/ARDS 342,345–50

acute lupus pneumonitis 815amiodarone toxicity 590bleomycin toxicity 592connective tissue disease 804dermatomyositis 826drug-induced lung disease

586–7, 589exudative phase 379–80, 380fibroproliferative phase 348histological features 381hyaline membrane 186influenza 189–90, 189intravascular thrombosis 58late stage 381measles 193mixed connective tissue

disease 828

organizing phase 349, 379–80,381, 587

parainfluenza viruses 192paraquat toxicity 598patchy, discrete 382pathology 349, 380, 380–1polymyositis 826proliferative phase 380–1radiological findings 379respiratory syncytial virus 191rheumatoid arthritis 811, 811SARS 190squamous metaplasia 348systemic lupus erythematosus

815, 816with thrombus 347viral infections 184, 186–7

diffuse alveolar hemorrhageantiphospholipid syndrome

816capillaritis 816clinical features 815–16differential diagnosis 817histopathology 816rheumatoid arthritis 813systemic lupus erythematosus

815–17, 815, 816diffuse idiopathic pulmonary

neuroendocrine cellhyperplasia (DIPNECH)21, 889, 1151, 1173–6

carcinoid tumors 1176clinical features 1173–4gender 1153histopathology 1174–6immunohistochemistry 1174,

1176incidence 1173neuroendocrine cell

proliferation 1177–8reactive 1176

pathology 1174, 1175peribronchiolar fibrosis 1176pulmonary neuroendocrine

cells 1175radiologic features 1173–4,

1174typical carcinoma in situ 1176

diffuse large B-cell lymphoma(DLBCL) 1330–1, 1346

clinical features 1330diagnosis 1330–1immunohistochemistry 1331lymphomatoid granulomatosis

differential diagnosis 1335methotrexate-associated in

rheumatoid arthritis 814molecular pathology 1331pathology 1330, 1330–1primary effusion lymphoma


pyothorax-associatedlymphoma differentialdiagnosis 1357

diffuse panbronchiolitis (DPB)644, 645

differential diagnosis 644, 646etiology 644laboratory findings 644pathology 644

diffuse parenchymal lung disease366, 366

diffuse pleural fibrosis 1436–7,1444–8, 1447

asbestos fiber counts 1447calcifying fibrous tumor of the


chest pain 1445–6, 1448clinical manifestations 1445–6differential diagnosis 1447–8drug-induced 1445etiology 1445fungal infections 1447genetics 1444histology 1446–7incidence 1444lag period from asbestos

exposure 1446lung function restriction 1448macroscopic pathology 1446pathophysiology 1448pleural plaque differential

diagnosis 1442pleuropulmonary malignancy

risk 1448progression/prognosis 1448radiographic findings 1446retroperitoneal fibrosis 1445tuberculosis 1447

diffuse pulmonarylymphangiomatosis1258–61


1259differential diagnosis 1259–60histopathology 1259immunohistochemistry 1259lymphangioleiomyomatosis


macroscopic pathology 1259,1260

natural history 1260–1prognosis 1260–1radiographic findings 1259treatment 1261

diffuse pulmonarymeningotheliomatosis1228

Dirofilaria (dirofilariasis) 311–14clinical features 311–12coin lesion 312dead parasites 313diagnosis 313epidemiology 311

Index

1576






life cycle 312organism 311pathology 312–13, 313radiographic features 312subcutaneous 322treatment 314

disseminated intravascularcoagulation (DIC),pulmonary 58

disseminated juvenilexanthogranuloma 126

DNA aneuploidy, squamousdysplasia/carcinoma-in-situ 907

DNA methylation, mesothelialhyperplasia 1454

DNA sequences, mycobacterialinfections 208–9

Doege–Potter syndrome 1431Down syndrome, tracheal

stenosis 69Dressler syndrome 1420drug abuse 596–600cocaine abuse 596–7heroin toxicity 598–9intravenous 599–600lung transplantation lesions

790marijuana abuse 597–8pulmonary artery embolism

684see also intravenous drug

abusedrug-induced lung disease

585–600amiodarone toxicity 590, 590Niemann–Pick diseasedifferential diagnosis 416

nodules 591biopsy 585–6bleomycin toxicity 591–2busulfan toxicity 591, 592chronic interstitial

pneumonitis 587–8diagnosis 585diagnostic criteria 585diffuse alveolar damage

586–7, 589diffuse pleural fibrosis 1445eosinophilic pneumonia 588filler substance histologic

characteristics 599granulomatous inflammation

589granulomatous pneumonitis 465histologic pattern 585–90infliximab toxicity 593, 593interstitial in hypersensitivity

pneumonitis differentialdiagnosis 465

KL-6 levels 585lung transplantation 790lymphomatoid granulomatosis

1332lupus 589–90, 819, 1426–7

methotrexate toxicity590–1,591drug-induced lymphoma814, 1345

monoclonal antibody toxicity592–3

nitrofurantoin toxicity 592nonspecific interstitial

pneumonia 587–8organizing pneumonia 588pathogenesis 586patterns of injury 587pleural disease 589–90, 1412,

1426–7plexogenic arteriopathy 673pulmonary alveolar

proteinosis 589pulmonary edema 589pulmonary hemorrhage 589, 742pulmonary hypertension 589rapamycin/rapamycin analog

toxicity 592sirolimus toxicity 592, 791, 1255systemic lupus erythematosus

819tyrosine kinase inhibitor

toxicity 592, 593usual interstitial pneumonia

587–8vasculitis 589veno-occlusive disease 589

drug reactions, sarcoidosisdifferential diagnosis 492

dysplasia see squamous dysplasia/carcinoma-in-situ(SD/CIS)

Duffy blood group 297dyspneadiffuse pleural fibrosis 1445pleural effusion 1410solitary fibrous tumor of the

pleura 1435dystrophic ossification 54

E-cadherin 1495mesothelioma diagnosis 1499sarcomatoid mesothelioma

1503echinocandins 239, 244echinococcosis 328–34Echinococcus granulosus hydatid

lung disease 328–31alveolar 332antibody detection 330clinical features 328–9cytology 331diagnosis 330–1differential diagnosis 330–1epidemiology 328hooklets 332hydatid cyst 328–34, 330, 331immunodiagnostic tests 330life cycle 329organism 328pathology 329–30protoscolices 331

radiographic appearance 330Echinococcus multilocularis

hydatid lung disease 332–3clinical features 332diagnosis 332epidemiology 332organism 332pathology 332treatment 332–3

echinoderm microtubule-associated protein-like4–anaplastic lymphomareceptor tyrosine kinasesee EML4-ALK fusion gene

echovirus 118Ecstasy abuse 600ectopic pulmonary deciduosis

876, 877, 878ectopic tissue in lungs 90–1, 91efferent autonomic nerves 12EGFR gene mutations

see endothelial growthfactor receptor (EGFR)gene mutations

Ehlers–Danlos syndrome 429–32classification 430clinical features 430epidemiology 429genetics 430laboratory findings 431parenchymal tears 430–1pathology 430–1, 431pathophysiology 431pleural fibrosis 1445primary spontaneous

pneumothorax 1415prognosis 431radiological findings 430, 430treatment 431–2vascular type 430, 431

Ehrlichia chaffeensis pneumonia179

Eisenmenger’s syndrome,plexogenic arteriopathy673

elastic fibers, pigmentation 58elastic recoil, aging effects 32elastin, absence in pulmonary

hypoplasia 93electron microscopy (EM) 50embolism 112EML4-ALK fusion gene 1191translocation in lung

adenocarcinoma 1063,1066

Emmonsia parvasee adiaspiromycosis

emphysemaA1AT deficiency 619–20adaptive immune response 621alveolar septal fibrosis 386bullous 615, 618centriacinar 615, 617centrilobular 616classification 615, 615–18, 616

congenital lobar 85–6, 85, 86definition 614development-related airspace

enlargement 623focal 615genetics 607homeostatic maintenance/

repair system disruption620–1

idiopathic pulmonary fibrosis608

immunological process 621irregular 615–18, 619localized giant bullous 624–5lung destruction 609lymphangioleiomyomatosis

1254, 1255macrophages 620Marfan syndrome 428methylphenidate abuse 600neutrophils 620non-destructive 623oxidant damage 620panacinar 615, 617paraseptal 615, 618pathogenesis 620–1placental transmogrification

626–7, 626proteolytic/antiproteolytic

imbalance 620pulmonary interstitial 109–11,

110radiological findings 608repair response after injury

620–1senile lung 623smoking 620, 621starvation 623–4vascular endothelial growth

factor function 620see also chronic obstructive

pulmonary disease(COPD)

empyema 1412, 1425Encephalitozoon

(microsporidiosis) 304,305, 305

Encephalitozoon cuniculi(microsporidiosis) 233,304, 304–5

clinical features 304–5differential diagnosis 305epidemiology 304organism 304pathology 305treatment 305ulcerative tracheitis 305

encephalomyelitis, lung cancer973–4

endobronchial metastases 1384–9diagnosis 1388–9gross pathology 1386histology 1386radiographic findings 1386treatment 1386–8

Index

1577






endocrinologic syndromes, lungcancer 972

endometrial carcinoma 1377metastases 1386, 1398

endometrial sarcoma, metastases1387

endometrial stromal sarcoma,metastases 1381, 1398

endometriosisperitoneal 1525pleural 1427–8, 1428

endometriosis, pulmonary 875–9clinical features 875clinopathological correlation

878cytology 877–8differential diagnosis 878, 878–9ectopic pulmonary deciduosis

876epidemiology 875etiology 875histopathology 875–6, 876, 877immunohistochemistry 878macroscopic pathology 875, 876natural history 879nodules 875, 876pathogenesis 878prognosis 879radiographic features 875, 875,

876treatment 879

endosalpingiosis 1525, 1525–6endothelial cellsactivation 353, 354–5acute lung injury 354–5, 355development 5

endothelial growth factorreceptor (EGFR)

atypical adenomatoushyperplasia 923–4

pulmonary adenocarcinoma 50pulmonary preinvasive

disease 905endothelial growth factor

receptor (EGFR) genemutation 924–5

lung adenocarcinoma 1062,1066

mesothelioma 1466mucoepidermoid carcinoma

1131pleomorphic carcinoma 1190–1pulmonary

adenocarcinogenesis 924endothelial growth factor

receptor (EGFR) genemutations 924, 924–5

endothelial growth factor receptor(EGFR) signaling pathway1062–3

endothelial growth factorreceptor (EGFR) tyrosinekinase inhibitors 1062

endothelins, pulmonaryhypertension 392

Entamoeba gingivalis 293–4Entamoeba histolytica

(amebiasis) 291–4, 292clinical features 293diagnosis 293differential diagnosis 293–4epidemiology 291–2organism 292–3pathology 293pleuropulmonary patterns 293treatment 294

enteric adenocarcinoma 1077–9enteric cysts 88–9Enterobacteriaceae, pneumonia

175–6Enterobius vermicularis

(enterobiasis) 320, 321Enterococcus pneumonia 176Enterocytozoon (microsporidiosis)

304, 305enterovirus 118environmental irradiation,

squamous dysplasia/carcinoma-in-situ 892

environmental pollutionasbestos 515, 1437–8, 1460chrysotile 1437, 1438erionite 1459lung cancer 951–2tremolite 1437–8, 1459, 1459–60

eosinophil(s)asthma 566chronic eosinophilic

pneumonia 569–70Churg–Strauss syndrome 734

eosinophilic bronchiolitis 644eosinophilic lung disease 563–80acute eosinophilic pneumonia

577–80allergic bronchopulmonary

fungal disease 573–5non-asthmatic eosinophilic

bronchitis 568see also asthma, chronic

eosinophilic pneumonia(CEP)

eosinophilic pleuritis 1352eosinophilic pneumoniaacute 588drug-induced lung disease 588rheumatoid arthritis 588, 814see also chronic eosinophilic

pneumonia (CEP)eosinophilic pneumonitisdesquamative interstitial


toxocariasis 319eosinophilic vasculitis,

pneumothorax differentialdiagnosis 1419

eotaxin 1412epidermal lysosomes, squamous

cell carcinoma 1103epithelial cell(s), CFTR 98

epithelial cell balls, asthma 567epithelial hyperplasia,

pre-invasive change 892epithelial membrane antigen

(EMA) 1430, 1489, 1494mesothelial hyperplasia 1453mesothelioma 1499, 1504–5

epithelial stem cells 7epithelial–mesenchymal

transition (EMT),sarcomatoid carcinoma1186–7

epithelial–myoepithelialcarcinoma 1136–8, 1136


1138cytology 1136–7, 1137differential diagnosis 1138electron microscopy 1137genetics 1136histopathology 1136immunohistochemistry 1137,

1137macroscopic pathology 1136molecular findings 1138myoepithelial cells 1136–7natural history 1138pneumocytic

adenomyoepitheliomadifferential diagnosis1145

prognosis 1138radiographic findings 1136

epithelioid cells, sarcoidosis 489epithelioid

hemangioendothelioma(EHE) 1263–8, 1265, 1514

cell of origin 1263–4classification 1263–4clinical features 1264, 1513clinicopathologic correlation

1264–6cytology 1264definition 1513diagnosis 1265–6differential diagnosis 879,

1266–7, 1435, 1513–4electron microscopy 1264histology 1513histopathology 1264immunohistochemistry 1264Kaposi’s sarcoma differential

diagnosis 1513–4macroscopic pathology 1264mesothelioma differential

diagnosis 1513–4molecular findings 1264natural history 1268nodules 1264prognosis 1268pulmonary endometriosis

differential diagnosis 879

radiographic findings 1264solitary fibrous tumor of the


treatment 1268epituberculosis, childhood 218Epstein–Barr virus (EBV) 198–9,

368cytotoxic drug-induced

lymphoma 1345endobronchial leiomyomatous

polyp 789inflammatory myofibroblastic

tumor 1275lung cancer 956lung transplantation 789lymphoepithelioma-like

carcinoma association1120

lymphoid interstitialpneumonia 1320

lymphomatoid granulomatosis199, 1334

lymphoproliferative disorders814

neoplasms 789NK/T-cell lymphoma, nasal

type 1335pneumonia 198–9, 200post-transplant

lymphoproliferativedisease 778, 786–9,1345

pulmonarylymphoepithelioma-likecarcinomas 956

pyothorax-associatedlymphoma 1356

Epstein–Barr virus (EBV)-associated B-celllymphoproliferativedisorders 1344

Erdheim–Chester disease 1353,1354, 1521

clinical features 1353Gaucher disease differential

diagnosis 412pathology 1353–4pleural 1522

erionite 538–9, 1459, 1459, 1461erlotinib 1066EGFR mutations 1062toxicity 593

Escherichia coli pneumonia 150,160, 175

esophageal atresia,tracheoesophagealfistula 70

esophageal development 4esophageal dysmotility, connective

tissue disease 804estrogen receptor(s) (ER)atypical adenomatous

hyperplasia 925lung adenocarcinoma 1066

Index

1578






estrogen receptor b,mesothelioma 1529

eukaryotic initiation factor 4E 925Ewing’s sarcoma 124, 124,

1376–7, 1519–20diagnosis 124

ex vivo lung perfusion (EVLP),lung transplantation 795

exogenous lipid pneumonia 632extra-corporeal membrane

oxygenation (ECMO),complications 111

extrapleural pneumonectomy,mesothelioma prognosisafter 1530

extremely-low gestational age,bronchopulmonarydysplasia 6

extrinsic allergic alveolitissee hypersensitivitypneumonitis

Fabry cells 413Fabry disease 417–18clinical manifestations 417cytology 418diagnosis 418electron microscopy 418, 418epidemiology 417genetics 417laboratory findings 418natural history 418pathology 417–18, 418pathophysiology 418prognosis 418radiological findings 417

factor V Leiden mutation 679–80Fallot’s tetralogy, tracheal

stenosis 68familial adenomatous polyposis

(FAP), sclerosinghemangioma association865

familial cryptogenic fibrosingpleuritis with Fanconi’ssyndrome 1445

familial Mediterranean fever,peritoneal mesothelioma1461

Fanconi’s syndromearteriovenous malformations

1261familial cryptogenic fibrosing

pleuritis 1445Farber bodies 426Farber disease 426–7clinical features 426electron microscopy 426epidemiology 426genetics 426laboratory findings 426pathology 426pathophysiology 426prognosis 426–7treatment 426–7

farmer’s lung 441, 442, 446, 467Fasciola hepatica (fascioliasis)

328FBN1 gene mutations 428feeding vessel sign 150–1ferruginous bodies, non-asbestos

518fetal adenocarcinoma 1073–7,

1170blastoma, differential diagnosis

1204carcinosarcoma differential

diagnosis 1204clinical features 1073cytology 1074differential diagnosis 1074,

1077electron microscopy 1077high-grade lesions 1073–8immunohistochemistry 1074–6low-grade lesions 1073, 1074morules 1074–6pathology 1073, 1074pleomorphic carcinoma


radiography 1074treatment 1077

fetal breathing impairment,pulmonary hypoplasia 91

fetal distress, meconium aspiratesyndrome 113

FHIT (fragile histidine triad) 906,1465–6

fibrillin-1 428, 429, 621fibroblast growth factor (FGF)

5, 5–6fibroblast growth factor 10

(FGF-10) 6fibroblast growth factor receptor

(FGFR) geneabnormalities 68

fibroepithelial polypssee bronchialinflammatory polyps

fibrohistiocytic neoplasms 1273–5fibrosarcoma, ameloblastic 1394fibrous glass 550fibrous webs, tracheal 71filarial infections 308–14clinical features 309diagnosis 310–11epidemiology 308organism 308–9pathogenesis 311pathology 309–10

fine-needle aspiration (FNA)41–2, 977

fissures 8fixationlarge specimens 46–7research techniques 47wedge biopsy 44–5

flavonoids, lung cancerprotection 959

fluconazole, Candida infectiontreatment 268

fluoro-edenite 1461folate 959–60folded lung 1436–7, 1448–51clinical manifestations 1448–9cytology 1450differential diagnosis 1451etiology 1449histopathology 1450–1macroscopic pathology 1450non-asbestos causes 1449prognosis 1451radiology 1449–50

follicular bronchiolitis 644, 646,810, 1318–9, 1319

benign pulmonary lymphoidproliferations 1318

clinical features 1318–9lymphoid hyperplasia 1318pathology 646, 1319prognosis 1319radiological features 1319rheumatoid arthritis 812–13

follicular dendritic cell sarcoma1352, 1352

follicular lymphoma 1331food flavorer’s lung disease 553foods, component effects on cell

function 959foreign bodygiant cell reaction 52–3, 686granulomas in sarcoidosis

differential diagnosis 492pulmonary embolism 686

foreign body-type cells fromaspiration, tuberculosisdifferential diagnosis 222

formalin fixation, wedge biopsy 45formalin inflation of lobes 46FOX genes, alveolar capillary

dysplasia 90fragile histidine triad (FHIT)

906, 1465–6Francisella tularensis 170pneumonia 170, 178

Fraser’s syndrome 87, 92frozen section 44–5wedge biopsy 44

Fryns syndrome, diaphragmatichernia 94

function of lungs 13–17fungal infections 226–78broncholithiasis 635diffuse pleural fibrosis 1447epidemiology 226–8histologic features 227HIV infection opportunist

infections 228incidence 227–8inflammatory myofibroblastic

tumor 1275lung carcinoma 957lung transplantation 774, 785organisms 226

pneumonia 147pulmonary hyalinizing

granuloma differentialdiagnosis 874

risk factors 227superinfection on viral

infections 185tuberculosis differential

diagnosis 221underlying diseases 227see also named infections and

organismsfungus balls 229, 232Scedosporium infection 243see also aspergilloma

Fusarium infection (fusariosis)243–4, 244

Aspergillus differentialdiagnosis 238

chlamydoconidia 244clinical features 244diagnosis 244epidemiology 243–4incidence 228organism 243pathology 244prognosis/treatment 244

galactocerebrosidasegalactosylceramideb-galactosidase 423

a-galactosidase A 417, 418GALC gene mutations 423ganglioneuroma 1519gastric adenocarcinoma,

metastases 1390gastrin-releasing peptide (GRP)

1165gastroesophageal reflux disease

(GERD) 466gastrointestinal autonomic nerve

tumor (GANT) 1511–2gastrointestinal pseudo-

obstruction 974gastrointestinal stromal tumor

(GIST) 1237metastases 1396–7, 1396solitary fibrous tumor of the


Gaucher cells 410–11, 411, 413,412, 700

conditions mimicking 412cytology 411electron microscopy 411

Gaucher disease 409–14, 700clinical manifestations 409–10cytology 410, 411, 411differential diagnosis 412–13enzyme replacement therapy

413–14epidemiology 409, 410genetics 409interstitial lung disease 410laboratory findings 411

Index

1579






Gaucher disease (cont.)natural history 413–14pathology 410–11, 411pathophysiology 411–12prognosis 413–14pulmonary hypertension

410, 412, 698enzyme replacementtherapy 414

radiological findings 410type 1 409, 410, 410, 412type 2 409, 410type 3 409, 410

Gaucher-like cells, GM1gangliosidosis 423

GCDFP-15, lungadenocarcinoma 1066

gefitinib 1062, 1066genomic instability, pulmonary

preinvasive disease907–8

Geotrichum capitatum infection228

gestational trophoblastic disease,metastases 1398–9

giant cell arteritis 745–7cellular immunity 746clinical manifestations 746differential diagnosis 747disseminated visceral 747epidemiology 745genetics 746histopathology 746humoral immunity 746infections 746laboratory findings 746natural history 747pathogenesis 746pathology 746prognosis 747Takayasu arteritis differential

diagnosis 747, 747, 748giant cell carcinoma 1196giant cell pneumonia 193giant cell sarcoma 1195–7clinical features 1196cytology 1197definition 1195–6differential diagnosis 1197electron microscopy 1197histopathology 1196–7immunohistochemistry 1197inflammatory stroma 1196–7macroscopic pathology 1196natural history 1197prognosis 1197smoking 1196

giant cell tumor 1197, 1275, 1276,1376–7

glandular inclusions, benign 1393glandular papilloma 856, 1065mixed squamous cell

papilloma 856, 857glass fiber 550glial tissue, ectopic 91

glioblastoma multiforme,metastases 1387

globoid leukodystrophysee Krabbe disease

glomangiosarcoma 1248, 1249Glomeromycota infection 228glomus tumors see primary

pulmonary glomus tumors‘gloved finger’ sign, allergic

bronchopulmonaryfungal disease 574

glucocerebrosidase genemutations 409

GLUT1 glucose transporter,mesothelioma diagnosis1500

glutathione-S-transferase (GST)950, 951

gene polymorphisms 519, 891,1440

glycogen storage disease, type II425–6

glycosaminoglycans 421, 422, 423glycosphingolipid 417–18GM1 gangliosidosis 423clinical manifestations 423natural history 423pathology 423, 424prognosis 423

Gnathostoma spinigerum(gnathostomiasis) 321–2

goblet cells 15, 18hyperplasia 892, 892metaplasia 565

Goodpasture syndrome 736–40acute interstitial pneumonia

differential diagnosis 382ANCA 739anti-basement membrane

antibodies 738, 739autoantibodies 739clinical manifestations 736–7cytology 737differential diagnosis 740epidemiology 736genetics 736hemoptysis 736–7hemosiderin-laden

macrophages 737immunohistochemistry 738, 739laboratory findings 738natural history 740pathogenesis 739–40pathology 737, 738prognosis 740pulmonary endometriosis

differential diagnosis 879radiographic findings 737, 737relapse rates 740ultrastructural studies 737Wegener’s granulomatosis

overlap 739–40Gorham–Stout syndrome

1428–9Gottron’s papules 825

Gram-negative bacteria,pneumonia 148

granular cell tumors 1239–42cell of origin 1240classification 1240clinical features 1240clinicopathologic correlation

1242cytology 1242differential diagnosis 1242electron microscopy 1242histopathology 1241, 1241–2immunohistochemistry 1242macroscopic pathology 1240–1,

1241malignancy 1242molecular findings 1242multiple endobronchial 1240–1natural history 1242prognosis 1242radiographic findings 1240, 1240

granulation tissue, Kaposi’ssarcoma differentialdiagnosis 1272

granulocyte–macrophagecolony-stimulating factor(GM-CSF)

Langerhans cell histiocytosis1351

lung cancer 975pulmonary alveolar

proteinosis 395, 397granulocytic sarcoma 1347granulocytosis 975granulomasallergic in Churg–Strauss

syndrome 734, 735hyalinizing 1430infectious 729necrotising 219pleural calcified 1430tuberculous type 218–19see also sarcoid granulomas

granulomatous disorders 464–7pleural disease 1423–5see also bronchiolitis,

granulomatous,bronchocentricgranulomatosis,necrotizing sarcoidgranulomatosis,pulmonary hyalinizinggranuloma (PHG),Wegener’s granulomatosis

granulomatous inflammation222, 589

Wegener granulomatosis724–5, 726–7, 728

granulomatous lymphadenitis 635granulomatous pleural disease

1423–5granulomatous pneumonitis

465, 466granulosa cell tumors of ovary,

metastases 1398

graphite miners’ lungs 536Grimelius technique 1164gross cystic disease fluid protein

(GCDFP-15), lungadenocarcinoma 1066

ground-glass nodules (GGN),lung adenocarcinoma1048–9, 1048

Group B streptococcal infection115

hyaline membrane disease105, 105

pneumonia 115–16growth factors 5, 1466–7growth of lungpulmonary neuroendocrine

cells 20sex effect 6stages 1trachea 6

H1N1 influenza 182, 184acute bronchiolitis 640antigenic shift 187infection spread 188pandemic 184pathology 190risk factors 188

Haemophilus influenzae 160, 161cystic fibrosis 99pleural exudate 160pneumonia 147, 176

halo signhemorrhage in metastases 1378invasive aspergillosis 235, 244reversed in mucormycosis 240

Hamazaki–Wesenberg bodies59, 59

sarcoidosis 486, 486Hamman–Rich syndrome

see acute interstitialpneumonia (AIP)

Hand–Schüller–Christian disease1348

Hantavirus 182hemorrhagic fever 199

hantavirus pulmonary syndrome199–200, 200

hard metal lung disease 541–3clinical presentation 542cytology 542diagnosis 542–3differential diagnosis 543giant cell interstitial

pneumonitis 543pathogenesis 542pathology 543, 544radiographic findings 542susceptibility 542

Harlequin sign 970, 970hASH1 transcription factor 1165HBME-1 1499, 1503head and neck cancer 946, 1393metastases 1393, 1394

heart, development 4

Index

1580






heat-shock proteins (Hsp) 751, 906Hecht’s giant cell pneumonia 193hedgehog interacting protein

957helminths 288, 308–35cestodes 328–35filarial infections 308–14nematodes 308–22pulmonary eosinophilia

syndromes 308soil-transmitted 314–18trematodes 322–8

hemangioendotheliomasee epithelioidhemangioendothelioma

hemangiomas 1263, 1263differential diagnosis 1263pathology 1263

hematite 543–4hematite miner’s lung 543–4complications 544

hematologic syndromes, lungcarcinoma 972, 975

hemoptysisBehçet syndrome 750Goodpasture syndrome 736–7pulmonary endometriosis 875

hemorrhagic fever, Hantavirus199

hemosiderin, biopsy artefact 51–2hemosiderosis see idiopathic

pulmonaryhemosiderosis

Hennekam syndrome 87hepatic cirrhosis, pleural effusion

1411hepatitis C virus, primary

effusion lymphoma 1355hepatocellular carcinoma,

metastases 1395–6hepatocyte growth factor,

mesothelioma 1467HER2 gene 905, 924hereditary hemorrhagic

telangiectasia,arteriovenousmalformations 1261, 1262

Hering Breuer reflex 1436Hermansky–Pudlak syndrome

418–21ceroid storage 421clinical manifestations 419cytology 420, 421differential diagnosis 421electron microscopy 421, 421epidemiology 418–19genetics 419natural history 421pathology 419–21, 419, 420pathophysiology 421prognosis 421pulmonary fibrosis 419, 421radiological findings 419, 419

heroin toxicity 598–9pathology 598–9

herpesvirus (HSV) 117, 184,195–7, 197

ARDS 196bronchiolitis 186diagnosis 196, 197, 198immunocompromised

patients 198immunosuppression 196–7inclusions in squamous

respiratory epithelium199

lung transplantation 784–5neutrophilic exudate 197pneumonia 117, 196polykaryons with nuclear

inclusions 197radiographic appearance 196respiratory mucosa 196see also Epstein–Barr virus

(EBV), varicella zostervirus (VZV)

high-frequency jet ventilation(HFJV), complications 111

high-resolution computedtomography (HRCT),lung carcinoma 965

hilar lymph nodescystic fibrosis 103metastatic pulmonary nodular

disease 1393hilar lymphadenopathy 1393histiocytic disorders 1354crystal-storing histiocytosis

412, 413see also Langerhans’ cell

histiocytosishistiocytic sarcoma 1352histiocytosiscrystal-storing 1341Erdheim–Chester disease

1354Rosai-Dorfman disease 1354

histiocytosis X 1348histochemical stains 49histological processinglarge specimen 43–5small specimen 42–3

histology of lungs 13–17Histoplasma capsulatum

var. capsulatum(histoplasmosis) 228,245–50, 245

acute disease 245–6, 247antigen detection test 249Candida differential diagnosis

267–8chronic cavitary 246, 246, 247cicatricial 246, 247clinical features 245–6coccidioidomycosis differential

diagnosis 262complement fixation test 249culture 247–8diagnosis 247–50differential diagnosis 249–50

epidemiology 245folded lung differential

diagnosis 1451forms 245, 246immunodiffusion test 249latent disease 246latex agglutination test 249leishmania differential

diagnosis 290–1lymphomatoid granulomatosis


organism 245pathogenesis 245pathology 246–7PCP differential diagnosis 272progressive disseminated

246–7, 248serological tests 248–9South American blastomycosis

differential diagnosis 264toxoplasmosis differential

diagnosis 303treatment 250

Histoplasma capsulatum var.duboisii (Africanhistoplasmosis) 249–50

HIV/AIDS-related lymphoma1344

HIV infection 200–2amebiasis 294BMPR2 expression inhibition

670Candida infection 265cavitary rheumatoid nodules

807CMV infection 195, 196cryptococcosis 254Kaposi sarcoma 1268, 1272leishmaniasis 290lung cancer 956lymphoid interstitial

pneumonia 264, 1319,1320

lymphomatoid granulomatosis1332

microsporidiosis 304nonspecific interstitial

pneumonia 266opportunistic infections 182,

228Pneumocystis jiroveci

pneumonia 268, 269primary effusion lymphoma

1355pulmonary abnormalities 201sarcoidosis 490South American blastomycosis

263tuberculosis coinfection 206,

209, 211, 223virus 262

HMB-45 immunostain,lymphangioleiomyomatosis 1253

Hodgkin lymphoma 1342, 1347clinical features 1342cytology 1342differential diagnosis 1342lymphocyte predominant 1347lymphomatoid granulomatosis


pathology 1342, 1342primary effusion lymphoma


pyothorax-associatedlymphoma differentialdiagnosis 1357

hookworm infection 316, 316horseshoe lung 75, 75host defensebacterial infections 153barriers 153foreign material recognition 153pneumonia 152–4

hot-tub lung 211, 219–20, 220,458–9

granulomas 458–9, 458histopathology 458–9presentation 458radiological findings 458treatment 459

HPS gene mutations 419Hughes–Stovin syndrome 751–2,

832b-human chorionic

gonadotrophin (b-hCG),pleomorphic carcinoma1190

human herpes virus 6 (HHV-6)199, 784, 1351

oncogenesis mechanism 1271–2human herpes virus 7 (HHV-7)

784human herpes virus 8 (HHV-8)

1268detection 1271inflammatory myofibroblastic

tumor 1275latent infection 1272primary effusion lymphoma

1355human metapneumovirus 191human milk fat globulins

(HMFG), mesothelioma1499

human papilloma virus (HPV)lung cancer 953–6squamous cell carcinoma 906,

1098–9squamous cell papillomas

850, 852–3, 855transmission route 956

human T cell lymphotropic virus1 (HTLV-1) 316, 644

humoral factors, viral infections184

humoral immunity 735, 746

Index

1581






Hunter syndrome 421, 422Hurler syndrome 421, 422Hurler–Scheie syndrome 422hyaline membrane disease

103–6, 104, 107bronchopulmonary dysplasia

106epithelial–endothelial injury

105–6, 105Group B streptococcal

infection 105, 105ischemia 105–6prematurity-associated 105–6term presentation 105–6thyroid transcription factor 1

1016ventilatory therapy 106see also respiratory distress

syndromehyaline membranesacute eosinophilic pneumonia

579acute lung infection/ARDS

345–6, 347diffuse alveolar damage 186

hyalinizing granuloma 1430hydatid cyst 328–34, 330, 331degenerate 331see also Echinococcus granulosus

hydatid lung diseasehydatid lung disease

see Echinococcusgranulosus hydatid lungdisease, Echinococcusmultilocularis hydatidlung disease

hydatiform mole, invasive 1399hydrocele, benign reactive

mesothelial proliferations1472–3

hydrops fetalis, congenitalperibronchialmyofibroblastic tumor 124

5-hydroxytryptamine (5-HT)see serotonin

hyperacute rejection, lungtransplantation 768,771–2, 773

hypercalcemia, paraneoplasticsyndrome in lungcancer 971

hyperinfection syndrome,strongyloidiasis 317

hyperinflation of lungs 623hypersensitivity pneumonitis

367, 371, 439–67acute 443, 450acute exacerbations 441, 458airspace organization 453antigens 441, 442, 446avoidance 467

aspergillosis 230bronchiolitis 450, 455bronchiolitis obliterans

organizing pneumonia 455

bronchoalveolar lavage 442–3carbon monoxide diffusion

capacity 442centrilobular fibrosis 455–7chronic 442, 443, 454, 455, 456,

457, 458acute exacerbations 458immunological pathways 449Japanese summer-type 459pathological findings 454–8radiological findings 444–6

classification 439, 440clinical features 439–43clinical testing 441–3consort 441diagnostic criteria 439differential diagnosis 459–67,

460early/subacute 450, 451etiology 446–9, 447fibrosis 455–7, 457honeycomb 457

fibrotic 441, 445–6forms 458–9gene analysis 446–9gene expression patterns 449granulomas 451–3, 454hot tub lung 458–9

ground-glass opacification443–444, 444, 445, 446

hard metal lung diseasedifferential diagnosis 543

immunological pathways446–9

immunological testing 442incidence 439–41inciting agents 446inflammatory pathway 449inhalational challenge 443interstitial lung disease 441interstitial pneumonitis 451,

455intraluminal fibrosis 453, 455isocyanate-related 459Japanese summer-type 459lymphangioleiomyomatosis


lymphoid proliferation 1318metal-working fluids 459, 467microchimerism 449mucostasis 454neutrophilia 443nonspecific interstitial

pneumonia pattern 378pathogenesis 446–9pathological findings 449–58,

453, 1318pathophysiology 441–2prevalence 439–41prognosis 467provocation tests 443pulmonary function testing

441–2radiological findings 443–6

sarcoidosis differential diagnosis491

skin testing 443smoking effects 449subacute 443, 451, 452, 453immunological pathways449

pathological findings 450–3phase 442radiological findings 443–4

subacute/chronic 451, 452survival 467treatment 467vasculopathy 457–8

hypersensitivity-type disease219–20

hypertrophic pulmonaryosteoarthropathy(HPOA) 973

solitary fibrous tumor of thepleura 1431

hypoglycemia, paraneoplastic1431

hyponatremia of malignancy972

hypoxemic hepatopulmonarysyndrome 325

hypoxiasickle cell disease 680

hypoxic arteriopathy 686–9, 690associated conditions 689causal factors 686–9COPD 688epidemiology 686–9histopathology 689intimal fibrosis 689medial hyperplasia 689pulmonary artery adventitial

thickening 689vascular lesions 689

idiopathic crescenticglomerulonephritis,autoantibodies 711

idiopathic interstitial pneumonia366, 464, 806

idiopathic lymphocytic pleuritis(ILP) 1449

idiopathic mediastinal fibrosis1447–8

idiopathic pleuroparenchymalfibroelastosis 390–1, 1423

idiopathic pulmonary arterialhypertension (IPAH),lung transplantation 768

pediatric 768idiopathic pulmonary fibrosis

(IPF)acute exacerbation 383–5clinical presentation 383diagnostic criteria 383differential diagnosis 384natural history 385pathogenesis 384pathology 383, 384

prognosis 385progression 384radiological changes 383

endothelin-1 392hypersensitivity pneumonitis

differential diagnosis 449lung cancer association 957–8malignancy association 372pathology 369, 370pulmonary hypertension 392usual interstitial pneumonia

pattern 371, 372see also usual interstitial

pneumonia (UIP)idiopathic pulmonary

hemosiderosis 742, 743,879

illicit drug abuse 596–600cocaine abuse 596–7heroin toxicity 598–9intravenous 599–600lung transplantation lesions

790marijuana abuse 597–8

imatinib 593immune anatomy 185immune complex deposition,

systemic lupuserythematosus 817

immune dysfunction, silicosis531–2

immune reconstitutioninflammatory syndrome(IRIS) 490

immune responseCandida infection 264histoplasmosis infection 245innate 154

immunocompromised patientsacute bronchiolitis 640acute bronchopneumonia 150adenovirus 193amebiasis 294Aspergillus infection 229babesiosis 300blastomycosis 199Candida infection 265coccidioidomycosis 260,

261, 262cryptococcosis 251–2cytotoxic drug-induced

lymphoma 1345herpesvirus 198histoplasmosis 245–6HIV/AIDS-related lymphoma

1344influenza severity 189lymphoma in congenital

immunodeficiency 1344lymphomatoid granulomatosis

1332malignant lymphoma 1344microsporidiosis 304Pneumocystis jiroveci

pneumonia (PCP) 268–9

Index

1582






Scedosporium infection 243South American blastomycosis

263sporotrichosis 272strongyloidiasis 316toxoplasmosis 303see also HIV infection,

post-transplantlymphoproliferativedisease (PTLD)

immunodeficiency, congenital 1344immunoglobulin A (IgA) 644immunoglobulin A, secretory

(sIgA) 184immunoglobulin (Ig) deposition

disease 1340immunoglobulin G 4 (IgG4)-

related sclerosing disease1420, 1421, 1520

immunohistochemistry 49–50argentaffinity 1164differential diagnosis problems

1029extra-pulmonary tumors 1030lung tumorscytokeratins 1027diagnosis 1015Napsin A 1023neuroendocrine markers1026, 1027

p16 protein 1027p63 protein 1027rapid technique for frozensections 1023

surfactant protein A 1025,1026

surfactant protein B 1025,1026

see also thyroid transcriptionfactor 1 (TTF1)

immunosuppressionCMV 195diffuse pneumonic

aspergillosis 237HSV infection 196–7iatrogenic 182lung cancer risk 956suppurative aspergillosis 237tracheobronchial aspergillosis

237immunosuppressive drugs, organ

transplantation 1348IMP3 (insulin-like growth

factor II messengerribonucleic acid-bindingprotein 3) 1505

inclusion cysts, peritoneal1522–3, 1523, 1524

infantsneuroendocrine cell hyperplasia

114–15, 641, 1172Pompe disease 425–6preterm 107, 108–9pulmonary alveolar

proteinosis 395

pulmonary vascular anatomy666

see also perinatal lungpathology, persistentpulmonary hypertensionof the newborn

infectionsacute bronchiolitis 638–9, 640acute cellular rejection

morphological mimics 777acute interstitial pneumonia

differential diagnosis 382acute lung injury/ARDS 350,

357trigger 343

amniotic fluid 115ascending 115Behçet’s syndrome 751bronchiolitis 638–9, 640, 151chronic bronchitis in COPD 612cystic fibrosis 99–100fetal 115folded lung differential

diagnosis 1451giant cell arteritis 746granulomatous bronchiolitis

645–6immunohistochemistry 49lung cancer 953–7lung transplantation 770, 774,

783, 783–9community-acquired 783early complications 773–4

lymphomatoid granulomatosisdifferential diagnosis1336

MALT lymphomas 1324mid-trimester miscarriage 115middle lobe syndrome 633–4nosocomial 773–4perinatal 115–18pleural effusions 1412pleural granulomas 1425polyarteritis nodosa 743–4pyoderma gangrenosum

differential diagnosis 832–3risk in Wegener

granulomatosis 715sarcoidosis 477, 488, 491scarring from healed

infection 54sickle cell disease 680Wegener’s granulomatosis

728, 729see also abscess, bacterial

infections, fungalinfections, viral infections

infectious granulomas 729infectious mononucleosis-like

PTLD 787inflammationbronchiectasis 629bronchiolar diseases with/

without adjacentparenchymal disease 643–8

COPD 611cystic fibrosis 99–100granulomatous 222, 589Wegener’s granulomatosis724–5, 726–7, 728

IL-6 670lung cancer 957pulmonary hypertension 670

inflammation response, COPD623

inflammatory bowel disease833–5

ANCAs 834–5clinopathological patterns 834differential diagnosis 834–5prognosis 835

inflammatory cascade,bronchopulmonarydysplasia 108–9

inflammatory cells, acute cellularrejection morphologicalmimic 777

inflammatory myofibroblastictumor (IMT) 118–19,125–6, 1275–8, 1520–1

ALK gene association 1277calcifying fibrous tumor of the



1277–8cytology 1277differential diagnosis 1278electron microscopy 1277etiology 1275histopathology 1277immunohistochemistry 1277macroscopic pathology 1277molecular findings 1277natural history 1278pleomorphic carcinoma


pleural 1521prognosis 1278pulmonary hyalinizing

granuloma differentialdiagnosis 874

radiographic findings 1277spindle cell carcinoma


inflammatory polypsetiology 847pathology 848, 849radiological findings 848squamous cell papilloma

differential diagnosis 855inflammatory pseudotumor

1520–1inflammatory response, bacterial

infection 153–4

inflammatory sarcomatoidcarcinoma 1193–4, 1193

infliximab toxicity 593, 593influenzabacterial superinfection 189–90diffuse alveolar damage 189–90pneumonia 186, 189see also H1N1 influenza

influenza A 182–3, 187, 190antigenic drift/shift 187

influenza B 187influenza C 187influenza virus 187–90, 188clinical signs 189diagnosis 189disease severity 189hemagglutinin expression 188infectivity 189neuraminidase expression 188onset 189pandemic outbreak 187–8seasonal 189–90

inhalant abuse 600inhalation pneumonia 149inhaled particles, aerodynamic

equivalent diameter 516inhibitor of apoptosis (IAP)

factors, atypicaladenomatoushyperplasia 923

iniencephalus, lung herniation 75injurylung cancer risk 957–8pulmonary neuroendocrine

cell function 20–1insulin-like growth factor binding

proteins (IGFBP),lymphangioleio-myomatosis 1253

interferon therapy, sarcoidosis490, 492

interleukin 1b (IL-1b) 748interleukin 6 (IL-6) 670, 748, 975interleukin 8 (IL-8) 109interleukin 10 (IL-10) 732intermediate cells 15, 23interstitial emphysema 109–11,

110interstitial fibrosisALI/ARDS 347bronchiolitis 387diffuse 536

interstitial glycogenesis 114interstitial lung disease 113–15,

366–97acute exacerbation of

idiopathic pulmonaryfibrosis 383–5

acute interstitial pneumonia379–83

classification 367, 367connective tissue disease 378dermatomyositis 826desquamative interstitial

pneumonia 367, 388–91

Index

1583






interstitial lung disease (cont.)diffuse 113–15, 114, 378drug-related 465epidemiology 367–8Gaucher disease 410genetics 368hypersensitivity pneumonitis

441idiopathic pleuroparenchymal

fibroelastosis 390–1lung transplantation 768lymphoid proliferation 1317–8mixed connective tissue

disease 827polymyositis 826prognosis 819pulmonary calcification 392–3pulmonary hypertension 392,

698–700causes 699–700symptoms 699vasculopathy 698–9

pulmonary ossification 392,393

radiographic features 386respiratory bronchiolitis 385–8rheumatoid arthritis 808–9scleroderma 821–2Sjögren’s syndrome 823–4smoking-related 385surgical lung biopsy 366systemic lupus erythematosus

817–19treatment 819undifferentiated connective

tissue disease 829usual interstitial pneumonia/

idiopathic pulmonaryfibrosis 367, 368–74

associated conditions 369histologic features 372

see also diffuse alveolardamage (DAD),non-specific interstitialpneumonia (NSIP),respiratory bronchiolitis-associated interstitial lungdisease (RB-ILD)

interstitial pneumonia 463–4bronchiolocentric 467fibrosing 543chronic 492

see also idiopathic interstitialpneumonia, nonspecificinterstitial pneumonia(NSIP), usual interstitialpneumonia (UIP)

amiodarone toxicity 590drug-induced lung disease587–8

desquamative 114giant cell in hard metal lung

disease 543hypersensitivity pneumonitis

451, 455

intimal fibrosisconcentric laminar 675hypoxic arteriopathy 689plexogenic arteriopathy 674–5pulmonary capillary

hemangiomatosis 693pulmonary hypertension in

systemic sclerosis 697pulmonary veno-occlusive

disease 693intra-alveolar hemorrhagebiopsy artefact 51, 51–2real 52

intra-pulmonary lymph nodes(IPLN) 1316–7, 1317

diagnostic imaging 1316–7metastatic carcinoma 1316silicotic nodules 1316subpleural 12

intra-pulmonary solitary fibroustumor 874

intra-pulmonary thymoma1283–5cell of origin 1284classification 1284clinical features 1284clinicopathologic correlations

1285cytology 1284differential diagnosis 1285genetics 1284histopathology 1284immunohistochemistry 1284macroscopic pathology 1284molecular findings 1284–5natural history 1285prognosis 1285radiographic findings 1284

intrathoracic lymph nodesmetastatic spread 1391–4radiography 1393–4

intravascular large B-celllymphoma 1336, 1337

clinical features 1336differential diagnosis 1337immunohistochemistry 1337molecular pathology 1337pathology 1337plasma cell disorders 1337

intravascular lymphoma 1273intravascular thrombosis 58intravenous drug abuse 599–600filler materials 599, 599pulmonary artery embolism

153, 684ionizing radiation, mesothelioma

1461–2iron-associated disease 543–4, 544iron encrustation 58, 58ischemic heart disease, asbestosis

1437ischemic–reperfusion injury, lung

transplantation 770–1isocyanate-related

hypersensitivitypneumonitis 459

isolated tumor cells (ITCs),lung cancer 1012

Japanese summer-typehypersensitivitypneumonitis 459

juvenile-onset laryngealpapillomatosis 955

juvenile xanthogranuloma,disseminated 126

Kaempferol 959Kampmeier focus 32kaolin macule 538kaolin pneumoconiosis 538pathology 538

kaolinite 538, 1447kaolinosis 1445, 1447Kaposi’s sarcoma 1268–72, 1269cavernous–angiomatous 1271cell of origin 1268classification 1268clinical features 1268clinicopathologic correlation

1272cytology 1271differential diagnosis 1272diffuse pulmonary

lymphangiomatosisdifferential diagnosis1260

electron microscopy 1271epithelioid

hemangioendotheliomadifferential diagnosis1513–4

genetics 1269histopathology 1271HIV infection 1268, 1272immunohistochemistry 1271inflammatory 1271LANA-1 1271, 1272latent infection 1272macroscopic findings 1271molecular findings 1271–2natural history 1272organ transplantation 1268primary effusion lymphoma

1355primary pulmonary

angiosarcoma differentialdiagnosis 1273

prognosis 1272radiographic findings 1269–71spindle cells 1271, 1272treatment 1272

Kartagener’s syndrome 96Katayama syndrome 308, 324keratin genes, squamous cell

carcinoma 1104keratinocyte growth factor,

mesothelioma 1467Ki-67 proliferation index 1505Kirsten-rat derived retroviruses

causing sarcoma

mutations see KRASgene mutations

Klebsiella pneumoniaepneumonia 160, 175

Klippel-Feil syndrome 66, 75Krabbe disease 423–5clinical manifestations 423–4electron microscopy 424, 425macrophages 424pathology 424, 424prognosis 424–5radiological findings 424treatment 424–5

KRAS gene mutations 905atypical adenomatous

hyperplasia 924, 924–5localized non-mucinous

bronchioloalveolarcarcinoma 924

lung adenocarcinoma 924, 924,1062–3, 1068

lung carcinoma 951pleomorphic carcinoma 1190

Krebs von den Lunge-6 (KL-6)glycoprotein 585

Kveim–Siltzbach test, sarcoidosis489

lactate dehydrogenase (LDH)1410, 1529

Lady Windermere’s syndrome219–20, 220, 633–4

Lambert–Eaton syndrome 974,1153

Lambertosis see peribronchiolarmetaplasia

Langerhans cell(s) 1348, 1351Langerhans cell granulomatosis

(LCG) 1419Langerhans cell histiocytosis 1352advanced with pulmonary

hypertension 1351Birbeck granules 1348chronic eosinophilic


clinical features 1349clinicopathological

associations 1349cytokines 1351desquamative interstitial

pneumonia differentialdiagnosis 389–90

diagnosis 1349differential diagnosis 1351GM-CSF role 1351HHV-6 association 1351histogenesis 1351immunohistochemistry 49, 1350light chain deposition disease

differential diagnosis 1340lung transplantation 790lymphangioleiomyomatosis


Index

1584






malignant 1352multisystem 1352pathology 1350, 1351, 1351prognosis 1349progressive fibrotic 1350pulmonary hypertension 392radiology 1349smoking 1349, 1351treatment 1349

Langerhans cell histiocytosis(LCH) 385

Langerhans cell sarcoma 1352Langerin 1348, 1350lanthanides 544–5large amino acid transporter

type 1 (LAT1) 925large B-cell lymphomaintravascular 1336, 1337see also diffuse large B-cell

lymphoma (DLBCL)large cell carcinoma 1114–21cell of origin 1114classification 1114clinical findings 1114clinicopathologic correlation

1115–6cytology 1115, 1116differential diagnosis 1116–7electron microscopy 1115genetics 1115histopathology 1114–5immunohistochemistry 1115macroscopic pathology 1114,

1115, 1116molecular findings 1115, 1117natural history 1117paraneoplastic syndromes

1114primary pulmonary


prognosis 1117radiographic findings 1114,

1115rhabdoid phenotype 1121,

1122, 1242thyroid transcription factor 1

expression 1016, 1019variants 1117–21

large cell neuroendocrinecarcinoma (LCNEC)1019, 1027, 1161

adenocarcinoma component1162

atypical carcinoid tumordifferential diagnosis 1168

basaloid carcinoma differentialdiagnosis 1118


classification 1152clinical features 1153cytology 1161, 1161–2, 1168differential diagnosis 1167

electron microscopy 1166genetics 1166–7histopathology 1160–2immunohistochemistry 1165macroscopic pathology 1155natural history 1169pathology 1156prognosis 1169radiographic findings 1155,

1156small cell lung carcinoma

differential diagnosis1168–9, 1169

large pleural biopsy 49large surgical pathology specimen

processing 43–5, 47–9frozen section 44–5large pleural biopsy 49lung wedge biopsy 43–4surgical techniques 45–7

laryngeal atresia 87, 87laryngotracheobronchitis

see crouplate transforming growth

factor-b binding protein4 (LTBP4) 6

latency-associated nuclear antigen(LANA-1) 1271, 1272

latent TGF-b binding proteins(LTBP) 1467

Legionella, pneumonia 148,149, 176

Legionella micdadei 148, 161, 176Legionella pneumophila 148, 161,

162, 176leiomyoma/leiomyosarcoma

1242–3, 1255, 1382, 1520see also benign metastasizing

leiomyoma (BML)leiomyomatous hamartoma 1256leiomyomatous polyp 789Leishmania (leishmaniasis) 289–91amastigotes 216clinical features 290cutaneous 290diagnosis 290differential diagnosis 290–1epidemic 289epidemiology 289mucocutaneous 289, 290organism 289pathology 290pleural infection 291, 1425respiratory tract 290species 289sporadic 289transmission 289treatment 291visceral 289, 290

lepidic predominantadenocarcinoma (LPA)918, 1044, 1055, 1058–9

leprechaunism, development-related airspaceenlargement 623

Leptospira (leptospirosis)171, 178

Letterer–Siwe disease 1348, 1352leukemia 684–6, 1348, 1401leukotriene receptor antagonists,

Churg–Strauss syndrome735

Lewis antigen 1496Libby mine (Montana, US) 539–40Liebow’s bronchial probe

technique 46–7Li–Fraumeni syndrome 960light chain deposition disease

1340, 1340,differential diagnosis 1340variant 1340

Linguatula pentastomiasis 335lipidaspiration 632in capillaries 112

lipid pneumonia, endogenous/exogenous 412, 416

lipofuscin 418lipoma see primary pulmonary

lipomalipopolysaccharide 1409–10lipopolysaccharide-binding

protein (LBP) 1412liposarcomametastases 1384see also primary pulmonary

liposarcomaListeria monocytogenes 116, 116liver tumors, metastases 1395–6LKB1, atypical adenomatous

hyperplasia 925Loa loa (loiasis) 322lobar agenesis 75lobar consolidation, viral

infections 184lobar emphysema, diagnosis 76–7lobectomysimple 45–7sleeve 46

lobes, formalin inflation 46localized non-mucinous

bronchioloalveolarcarcinoma (LNMBAC)889, 911

atypical adenomatoushyperplasia

differential diagnosis 918distinction 927intermediate features 922mixed cell populations 922transition from 928

cell cycle activity 922collapsed pattern 919Dicer upregulation 927ground-glass opacity 928histologic features 918matrix metalloproteinases 926proliferation index 922

Löffler syndrome 308, 314–15,316, 317, 571

Löfgren syndrome 488, 492Lophomonas 307low-grade adenocarcinoma of the

fetal lung type (L-FLAC)/well-differentiated fetaladenocarcinoma (WDFA)1214

lower respiratory system 7, 14–15,150

lung adenocarcinoma 1043–79acinar subtype 1054age factors 1047apical 970atypical adenomatous

hyperplasia 919–21, 921,926

atypical carcinoid tumordifferential diagnosis1167

BRAF gene mutation 1063breast metastatic

adenocarcinomadifferential diagnosis 1031

bronchial mucosa 909carcinoma markers 1495–6cell of origin 1045central carcinoma 1051, 1051CK5/6 immunohistochemistry

1061classification 1043, 1043–5,

1044clinical features 1047, 1047clinopathologic correlation

1064colloid 1068–73epidemiology 1069immunohistochemistry 1070pathology 1069–70, 1070,1071

prognosis 1070colorectal metastatic

adenocarcinomadifferential diagnosis1031, 1032

copy number alterations1064

CT studies 1049cytokeratins 1028cytology 1056–61, 1060differential diagnosis 1059–61,

1065–6EGFR 50gene mutations 1062, 1066signaling pathway 1062

electron microscopy 1061EML4-ALK translocation

1063, 1066enteric pattern 1077–9, 1079epidemiology 1046–7epithelioid mesothelioma

immunohistochemicaldistinction 1494–5

ethnic group differences 1063extrathoracic manifestations

1047

Index

1585






lung adenocarcinoma (cont.)fetal 1073–7, 1076, 1077, 1078gender 1047gene amplifications/deletions

1064genetics 1045–6, 1045genomic imbalances 1045grading system 1056ground-glass nodules 1048–9,

1048histopathology 1051–6, 1066immunohistochemistry 1025,

1028, 1048, 1061, 1065–6incidence 889, 1046intrathoracic spread 1047invasive 1050, 1055, 1059mucinous 1066–8, 1067,1069, 1073

variants 1044–5, 1050–1,1055, 1057, 1058, 1059

karyotypic changes 1045, 1045KRAS gene mutations 924,

924, 1062–3lepidic predominant 918, 1044,

1055location 1047macroscopic pathology 1049–51mesothelioma 1491–2, 1494–5differential diagnosis 1494,1500, 1508–9

metastases 1028, 1047, 1049microinvasive 1054micropapillary predominant

1055minute pulmonary

meningothelial-likenodule association 1228

mixed subtype 1044, 1053molecular markers 1066molecular pathology 1061–4,

1062morphology 1053–4mucin globules 1476mucinous 1029, 1044, 1068,

1070differential diagnosis 1068epidemiology 1066invasive 1066–8, 1067, 1069,1073

KRAS mutations 1063, 1068prognosis 1068radiography 1066–7treatment 1068

mucoepidermoid carcinomadifferential diagnosis 1132

multifocal 1064, 1064natural history 1066nodules 1048–9, 1049non-smokers 1046–7, 1046non-terminal respiratory

unit 1045p63 immunohistochemistry

1061papillary predominant 1054–5parenchymal 910

pediatric 1047PET imaging 1049primary in children treated for

malignancy 1047prognosis 1066pseudomesotheliomatous 1051radiographic findings 1047–9,

1048, 1049screening 1047small cell lung carcinoma


smoking 1066solid predominant 1055squamous cell carcinoma

differential diagnosis1027, 1029

STK11/LKB1 gene mutation1063

subtypes 1045, 1046, 1053surfactant protein A 1025symptoms 1047tail sign 1047terminal respiratory unit 1045,

1046thyroid transcription factor 1

expression 1016, 1017,1061

treatment 1066typical carcinoid tumor


variants 1044–5, 1050–1,1066–79

visceral pleural invasion 1064see also adenocarcinoma

in situ (AIS), mucinouscystadenocarcinoma

lung adenosquamous carcinoma1063, 1093

lung agenesis 68, 74–5bilateral 74, 75unilateral 74–5

lung allocation score (LAS), lungtransplantation 767–8

lung cancer 945–79age 948alcohol ingestion 958a-1-antitrypsin deficiency 957apical 970arsenic exposure 952–3asbestos exposure 525–8, 953,

957diagnosis 526–7electron microscopy 526,527–8

risk 526smoking 525, 526tissue analysis 527–8

asbestos fiber burden 526asbestos fiber potency 525–6asbestosis association 525, 526asthma association 957autofluorescence

bronchoscopy 977

beryllium exposure 541bronchoscopy 976–7carcinoma with rhabdoid

phenotype 1171chest X-rays 965, 975–6chronic eosinophilic

pneumonia 572classification 889clinical manifestations 968–75coal workers’ pneumoconiosis

536connective tissue disease 804,

958COPD 957CREST syndrome 823CT scans 967, 968, 969, 976cytochrome P450 enzymes 949cytology 978dermatomyositis 827diagnostic methods 976–8diet association 958–60EGFR 50encephalomyelitis 973–4endocrinologic syndromes 972environmental pollution 951–2epidemiology 889, 945–50Epstein–Barr virus 956etiology 950–60familial 960, 965FDG-PET 976fine-needle aspiration 977flavonoid risk reduction 959folded lung differential

diagnosis 1451frozen sections 978gastrointestinal pseudo-

obstruction 974gender distribution 945, 947–8genetic risk 961genetic susceptibility 960–5genome-wide studies 963–5geographic trends 946hematologic syndromes

972, 975high-resolution computed

tomography 965histology 949–50HIV infection 956human papilloma virus 953–6hypertrophic pulmonary

osteoarthropathy 973hyponatremia of malignancy

972idiopathic pulmonary fibrosis

957–8immunohistochemistry 50immunosuppressed patients

956incidence 119, 889, 945–6infections 953–7inflammation association 957intraoperative palpation and

wedge resection 978intrathoracic spread 969–70invasive 901–2

isolated tumor cells 1012KRAS gene mutations 951Lambert–Eaton syndrome 974large specimen evaluation 47–9lung injury 957–8lymph node involvement 13,

970, 1007mediastinoscopy 977mesothelioma differential

diagnosis 1508–9metastases 970–1, 971, 1007,

1011mortality 945–6, 947MRI 976mucorales 239–41clinical features 240diagnosis 241differential diagnosis 241epidemiology 240organisms 239pathology 240prognosis 241treatment 241

multiple synchronous primarytumors 1012

mycobacterial infections 956–7neoadjuvant therapy 594–5nodules 1012non-smoking related 945–6occupational factors 952–3,

954, 955oxidative stress 958p53 gene mutations 951paraneoplastic syndromes

971–5, 972phytosterols 960pleomorphic and KRAS

mutations 1063pleura histologic sampling

1005pleural effusion examination

977pleural invasion 48, 1005polymyositis 827pre-existing lung lesions 890prognosis 978–9, 1013, 1020nodal disease 1011

race 948–9, 949radiation therapy with

chemotherapy 594–5, 595radiofrequency ablation 595radiography 975–6, 975,

976, 977radon exposure 951relapse 978–9rheumatoid arthritis 814rhinosinusitis 957risk after head and neck

cancer 946risk with pleural plaques 1443scleroderma 822–3screening 965–8, 966, 967, 969secondary pneumothorax

1415–6signs/symptoms 969, 969, 971

Index

1586






single gene polymorphisms960–3

smoking association 890–1,946, 947–8, 950–1

socioeconomic factors 950solid fuel use 946staging 48–9, 1004, 1007, 1009,

1010, 1011subacute sensory neuropathy

973–4survival 978, 1013talc miners 538TNM classification 1004carcinoid tumor 1012clinical 1004M category 1004, 1011, 1011multiple synchronousprimary tumors 1012

multiple tumor nodules1006, 1012

N category 1004, 1007, 1008original descriptors 1012pathologic 1004resection completeness 1013small cell lung cancer 1012specific situations 1013survival 1013T category 1004, 1005, 1006visceral pleural invasion1005

total burden 945traffic-related pollution 952treatment 978–9tuberculosis 956–7tumor extension 970tumor growth rate

determination 968tumor measurement 1005tumor nodules 1006tumor size 1005uranium miners 891, 953venous thromboembolism 975vermiculite-associated 539–40video-assisted thoracoscopic

surgery 977visceral pleural invasion 971,

1005vitamin A deficiency 958–9volume doubling time (VDT)

968welder’s pneumoconiosis 549see also lung adenocarcinoma

lung malformations 74–95classification 76congenital lobar emphysema

85–6congenital pulmonary

lymphangiectasis 87–8herniation 75pulmonary agenesis 74–5pulmonary cystic disease 75–82pulmonary hyperplasia 86–7pulmonary hypoplasia 91–3pulmonary sequestration

82–4

lung tissueabnormal 90–1ectopic 90–1, 91

lung transplantation 767–95adenovirus 784–5, 784airway anastomotic

complications 772–3airway inflammation without

scarring 780–1allograft rejection 769antibody-mediated rejection

768, 769, 781–3artificial lung technology 795Aspergillus infection 229, 785,

786bronchiolitis obliterans 768,

790–2bronchiolitis obliterans

syndrome 782–3, 790–2bronchoalveolar lavage 770bronchopneumonia 774Candida infection 785chronic vascular rejection

792–3, 794cytomegalovirus infection 777,

783–4, 784drug-induced lung disease 790early post-transplant period

770–4Epstein–Barr virus infection

778, 786–9ex vivo lung perfusion 795fungal infection 785graft failure 768, 772herpes simplex virus 784–5histological assessment 769–70hyperacute rejection 768,

771–2, 773illicit drug abuse 790indications 767, 768infections 770, 773–4,783, 783–9intermediate period after

774–90ischemic–reperfusion injury

770–1Langerhans’ cell histiocytosis

790late period 790–5lung allocation score 767–8lymphangioleiomyomatosis

793–5, 1255lymphocytic bronchiolitis

780, 781open lung biopsy 770pathologists’ role 768, 768–9pediatric population 768peri-operative period 770–4Pneumocystis jirovecii

pneumonia 785post-ischemic scarring 773primary graft dysfunction 770–1procedure types 767procedures performed 767pulmonary allograft

monitoring 769–70

recurrent disease in allograft793–5, 794

respiratory bronchiolitis 790smoking-related lesions

790, 791temporal approach to

pathology 770, 771transbronchial biopsy 769–70vascular anastomotic

complications 772–3viral infections 770, 783–5community-acquired 785

see also acute cellular rejectionin lung transplantation,post-transplantlymphoproliferativedisease (PTLD)

lung tumors 118–26antibodies, specificity 1015electron microscopy 50immunohistochemistry 50, 1015cytokeratins 1027Napsin A 1023, 1026neuroendocrine markers1026, 1027

p16 protein 1027p63 protein 1027, 1060, 1061rapid technique for frozensections 1023

surfactant protein A 1025,1026

surfactant protein B 1025,1026

immunoperoxidase indiagnosis 1015

incidence 119thyroid transcription factor 1

expression 1016staining 1023

lupusacute pneumonitis 815drug-induced 589–90, 819,

1426–7, 1427see also systemic lupus

erythematosus (SLE)lymph nodes 12AJCC/UICC Lung Cancer

Staging system 48–9,1007–11

aspirates 41–2, 1317–23hilar region of bronchi 9metastatic spread in

intrathoracic 1391–4see also intra-pulmonary

lymph nodes (IPLN)lymphangiectasia 76, 1259congenital 86, 87–8

lymphangioleiomyomatosis857–8, 1249–55, 1251

age factors 1250cell of origin 1249classification 1249clinical features 1250clinicopathologic correlation

1254

cysts 1250, 1252cytology 1253differential diagnosis 1254–5diffuse pulmonary

lymphangiomatosisdifferential diagnosis1259

electron microscopy 1253–4estrogen receptor positivity

1253extra-pulmonary 1252genetics 1249–50histopathology 1250–2, 1252HMB-45 immunostain 1253immunohistochemistry 1253insulin-like growth factor

binding proteins 1253light chain deposition disease

differential diagnosis 1340lung transplantation 793–5,

1255macroscopic pathology 1250matrix metalloproteinase

activity 1253micronodular pneumocyte

hyperplasia 1252molecular findings 1254natural history 1255progesterone receptor

positivity 1253prognosis 1255radiographic findings 1250secondary pneumothorax

1415, 1416treatment 1255TSC gene mutations 1249–50,

1254tuberous sclerosis complex

1249–50, 1252two-hit mechanism 1254

lymphangiomas 864, 1259, 1513lymphangiomatosis 1513see also diffuse pulmonary

lymphangiomatosislymphangitic carcinomatosis

1382–4lymphatic drainage of lung 12lymphatic dysplasia syndrome

1259–60lymphatic filariases 308–11clinical features 309diagnosis 310–11epidemiology 308organism 308–9pathogenesis 311pathology 309–10

lymphatic stomata 31, 1408, 1409lymphatics 11, 665see also lymph nodes

lymphocytic bronchiolitis 780,780, 781, 781

lymphocytic (lymphoid)interstitial pneumonia(LIP) 200–1, 367, 463,823, 1316, 1319–21

Index

1587






lymphocytic (lymphoid)interstitial pneumonia (LIP)(cont.)associated diseases 1319, 1319benign pulmonary lymphoid

proliferations 1318clinical features 1320connective tissue disease

association 463differential diagnosis 1320–1EBV infection 1320HIV infection 264, 1319, 1320hypersensitivity pneumonitis

differential diagnosis 463immunohistochemistry 1320lymphoid hyperplasia 1318marginal zone lymphoma


nonspecific interstitialpneumonia differentialdiagnosis 1321

pathology 1320, 1320prognosis 1320radiologic features 1320rheumatoid arthritis 811, 811Sjögren syndrome 823–4,

1319, 1320systemic lupus erythematosus

816treatment 1320

lymphocytic interstitialpneumonitis 804

lymphoepithelioma-likecarcinoma (LELC)1119–20

cytology 1119differential diagnosis 1119–20EBV expression 1120epidemiology 1119genetics 1119pathology 1119, 1120prognosis 1120

lymphohistiocytoidmesothelioma 1490

lymphoid hyperplasia 812–13,824, 825, 1322

lymphoid infiltrates 1028lymphoid tissue 1316–7lymphoma 126, 1324–45anaplastic large cell 1343B-cell lymphomas 1324–43,

1346age-related EBV-associated1344

common variableimmunodeficiency 1344

cytotoxic drugs 1345histogenesis 1324HIV/AIDS-related 1344immunocompromised patients

1344large cell carcinoma rhabdoid

phenotype differentialdiagnosis 1121

lymphoepithelioma-likecarcinoma differentialdiagnosis 1119–20

lymphoid hyperplasia 1318metastases 1401pleural 1354, 1514secondary 1357

secondaryclinical features 1345pleural 1357pulmonary changes 1345

T-cell lymphomas 1343, 1344,1346

see also specific namedlymphomas

lymphomatoid granulomatosis(LYG) 1331–6

classification 1334, 1334clinical features 1332EBV infection 199, 1334HIV infection 1332Hodgkin lymphoma


immunocompromised patients1332

immunohistochemistry 1334,1335

inflammatory infiltrate 1334pathology 1332, 1333, 1334,

1334treatment 1336vascular infiltration 1332Wegener’s granulomatosis

differential diagnosis 729lymphoplasmacytic lymphoma

(LPL) 1331lymphoproliferative disorders

126, 1316–57benign lymphoid

proliferations 1317–23classification 1316histiocytic disorders 1354rheumatoid arthritis 814see also lymphoma

lysinuric protein intolerance427–8

clinical manifestations 427electron microscopy 428epidemiology 427genetics 427laboratory findings 428pathology 427–8pathophysiology 428prognosis 428radiological findings 427, 427treatment 428

lysosomal storage diseases 409–27Fabry disease 417–18Farber disease 426–7Gaucher disease 409–14GM1 gangliosidosis 423Hermansky–Pudlak syndrome

418–21Krabbe disease 423–5

mucopolysaccharidoses 421–3Niemann–Pick diseasetype A and B 414–16type C 416–17

Pompe disease 425–6storage cells 413

Macleod’s syndrome 86macrophage mannose receptor

(MMR) 28macrophagesactivated exudate 185acute lung injury 353, 354ceroid-filled 420, 421chronic eosinophilic

pneumonia 569cigarette smoke-induced 385COPD 611desquamative interstitial

pneumonia 388desquamative interstitial

pneumonia-like reaction388

emphysema 620foamy 414–15, 416–17GM1 gangliosidosis 423hemosiderin-laden in

Goodpasture’s syndrome737

iron-laden 52Krabbe disease 424lysinuric protein intolerance 428marijuana abuse 597mycobacteria ingestion 212, 213respiratory fibrosis/RB-ILD

385, 386–7smokers 27smoking 385see also alveolar macrophages

malakoplakia 178–9, 412, 1242malaria 297–300clinical features 298diagnosis 299–300differential diagnosis 300drug toxicity 300Duffy blood group 297epidemiology 297histology 299life cycle 298organism 297–8pathogenesis 297, 299pathology 298–9, 299treatment 300

Malassezia furfur complex274–7, 276

catheter-associated 276clinical features 276diagnosis 277epidemiology 275–6pathology 276transmission 275–6

malignancyatypical mesothelial

hyperplasia distinction1453–4

connective tissue disease 804dermatomyositis 827idiopathic pulmonary fibrosis

association 372molecular evolution 929pleural exudate 1412polymyositis 827pulmonary artery embolism

684riskcongenital adenomatoidmalformations 79

diffuse pleural fibrosis 1448pleural plaques 1443

sarcoidosis-like granulomas491

sarcoidosis-like reaction 490Sjögren syndrome 825Wegener granulomatosis 715malignant

angioendotheliomatosissee intravascular largeB-cell lymphoma

malignant lymphomasee lymphoma

malignant melanomaclear cell tumor of the lung

differential diagnosis 1258large cell carcinoma rhabdoid

phenotype differentialdiagnosis 1121

lymphoepithelioma-likecarcinoma differentialdiagnosis 1120

mesothelioma differentialdiagnosis 1513

metastatic 1288, 1384, 1388,1391, 1401

diagnosis 1032, 1401differential diagnosis 1401intrathoracic lymph nodes1393

morphologic variability 1401treatment 1401

pleomorphic carcinomadifferential diagnosis 1194

pleural 1513primary pulmonary 1286–8,

1287, 1401primary pulmonary


spindle cell carcinomadifferential diagnosis 1194

malignant mesotheliomasee mesothelioma

malignant peripheral nervesheath tumor 1518–9

malignant small cell tumor of thethoracopulmonary region1170

malinosculation 76Mallory’s hyaline-like material

in type II pneumocytes56, 56

Index

1588






MAML2 gene mutations,mucoepidermoidcarcinoma 1131, 1132

Mammomonogamus laryngeus(mammomonogamiasis)322

man-made fibers 550–1dimensions 550–1epidemiology 551physicochemical

characteristics 551types 550see also refractory ceramic

fibers (RCF)manganese superoxide dismutase

(MnSOD) 1465maple bark stripper’s lung 453,

453Marfan’s syndrome 428–9, 1415clinical features 428electron microscopy 429epidemiology 428genetics 428pathology 429, 429pathophysiology 429prognosis 429radiological findings 428–9,

429treatment 429

marginal zone lymphoma 1318,1326, 1327

cytology 1329immunohistochemistry 1328plasmacytoma differential

diagnosis 1341Sjögren syndrome 825

marginal zone lymphoma,MALT-type 1324–9

amyloid deposition 1326chromosome translocations

1328clinical features 1324–5diagnosis 1325differential diagnosis 1328–9follicular pattern 1325immunohistochemistry 1326–7molecular pathology 1328pathology 1325–6plasma cell differentiation 1326radiographic findings 1325transformation 1326treatment 1329

marijuana abuse 597–8paraquat toxicity 598pathology 597, 597–8

Masson bodies 150, 809Masson–Fontana method 1164mast cell(s) 27, 28, 1283mast cell disease, systemic 1348matrixmetalloproteinases (MMPs)atypical adenomatous

hyperplasia 926localized non-mucinous

bronchioloalveolarcarcinoma 926

mesothelioma 1467preinvasive lesions 906–7

measles 192–3, 193pathology 193pneumonia 183, 193vaccination 193

mechanical ventilation 113air leaks 109, 111ball-valve effect 113

meconium aspiration syndrome112–13

clinical presentation 112definition 112fetal distress 113histopathology 112persistent pulmonary

hypertension of thenewborn association 113

ventilation/perfusionmismatch 112–13

meconium plug 112MECT1 gene mutations,

mucoepidermoidcarcinoma 1131, 1132

medial hyperplasiacongestive vasculopathy 690hypoxic arteriopathy 689plexogenic arteriopathy 674pulmonary capillary

hemangiomatosis 693pulmonary veno-occlusive

disease 693mediastinal lymph nodes,

metastatic pulmonarynodular disease 1393

mediastinal lymphadenopathy1393

mediastinal teratoma 1286pulmonary teratoma


mediastinal thymoma 1285mediastinum, mesothelioma

invasion 1469megakaryocytes 28, 28, 56–8melanoma see malignant

melanomamelioidosis 178Mendelian cancer syndromes,

lung cancer 960Mendelson’s syndrome 632meningiomametastases 1394see also primary pulmonary

meningiomamercury pneumonitis 550Merkel cell carcinoma of skin

1022, 1400mesenchymal neoplasms 1224–88angiomyolipoma 1255–6benign metastasizing

leiomyoma 1244–7diffuse pulmonary

lymphangiomatosis 1255,1258–61

granular cell tumors 1239–42minute pulmonary

meningothelial-likenodules 1227–30

neurogenic tumors 1239primary pulmonary

chondrosarcoma 1238–9primary pulmonary glomus

tumors 1247–9primary pulmonary leiomyoma/

leiomyosarcoma 1242–3primary pulmonary lipoma

1231–3primary pulmonary

liposarcoma 1233–4primary pulmonary

meningioma 1229,1230–1

primary pulmonary osteogenicsarcoma 1235–6

pulmonary angiomyolipoma1256

pulmonary chondroma 1237–8pulmonary hamartoma 1143–4,

1224–7pulmonary rhabdomyosarcoma

123–4, 1242pulmonary vein sarcoma1243–4see also clear cell tumor of the

lung (CCTL), lymphangioleiomyomatosis

mesenchymal stromal cells,lung-resident (LR-MSC) 4

mesenchymoma see pulmonaryhamartoma

mesothelial cellschemokine production 1412inclusions 1393inflammatory cell recruitment

1410malignant 1474pattern recognition receptors

1410pleural fluid 1408, 1409–10reactive 1474, 1475secretions 1409

mesothelial hyperplasia 32, 1419,1522, 1523

reactive 1453, 1453–4see also atypical mesothelial

hyperplasia (AMH)mesothelioma, atrioventricular

node 1473mesothelioma, malignant pleural

1454–522anatomic sites 1454apoptotic control 1465–6asbestos cell signaling 1464–5asbestos exposure 526, 1438,

1457–61asbestos fiber type 1456–7basal hemothorax 1477benign pleural disease

differentiation 1503–5,1504

biphasic 1480, 1486–7, 1487,1493, 1500–3, 1515–6

buildings with asbestos fibers1458

C-MET 1529calretinin sensitivity 1496–7carcinoma markers 1495–6CD10-positive 1509–10cdk inhibitors 1468chest pain 1468–9chest wall invasion 1476children 1460chromosomal changes 1467–8classification 1469, 1469–71clear cell 1488, 1488–9clinical features 1468–9cyclins 1468cytokeratin-negative 1496cytokeratins 1496, 1498cytology 1473–5, 1474D2-40 1498–9, 1509deciduoid 1487desmin 1505desmoplastic 1447, 1480, 1485,

1486diagnosis 50differential diagnosis 1502,

1508–22distant spread 1478–9E-cadherin 1499EGFR expression 1466electron microscopy 50, 1492–3environmental 1459–60epidemiology 1454–6epithelial membrane antigen

1499, 1504–5epithelioid 1479, 1480–2, 1492diagnosis 1493differential diagnosis 1508–15electron microscopy 1492–3histological subtypes 1481,1482, 1487–90

immunohistochemistry 1482immunohistochemistry inadenocarcinomadistinction 1494–5

well-differentiated papillarydifferential diagnosis1507–8

estrogen receptor b expression1529

familial 1460folded lung differential

diagnosis 1451giant cell 1489–90GLUT1 1500growth factors 1466–7HBME-1 positivity 1499hepatocyte growth factor 1467histochemical stains 1493–4histology 1480–92human milk fat globulins 1499immunohistochemistry 1475,

1494–5, 1504–5incidence 1438, 1455, 1456

Index

1589






mesothelioma, malignant pleural(cont.)intra-pulmonary spread 1478ionizing radiation 1461–2keratinocyte growth factor 1467Ki-67 proliferation index 1505lactate dehydrogenase 1529latent period from exposure to

asbestos 1464lepidic pattern 1490lipid-rich diffuse 1488localized 1477–8lung adenocarcinoma

differential diagnosis1065, 1494, 1500

lymphohistiocytoid 1491macroscopic pathology 1476,

1476–7markers 1501matrix metalloproteinases 1467mediastinal invasion 1469mesothelioma markers

1496–500metastases 1478–9microcystic 1487–8mortality ratio 1460mucin-positive 1488mucin staining 1493–4multicystic 1476myxoid 1477, 1483N-cadherin 1499nodules 1476non-asbestos causes 1461–3occupational exposure 1457–8oncogenesis mechanisms of

asbestos 1464P13-K/AKT pathway 1466p16/CDKN2A deletion 1529p53 tumor suppressor gene 1504paraoccupational 1458pathophysiology 1528PDGF 1466–7pericardial invasion 1476, 1477pleomorphic 1489, 1489–90pleural plaques 1442–3, 1476podoplanin 1499primary pulmonary osteogenic

sarcoma differentialdiagnosis 1236

prognosis 1528–30radiological findings 1469, 1470reactive oxygen species 1464–5refractory ceramic fibers 1462risk with pleural plaques 1443sarcomatoid 1479, 1482–5differential diagnosis 1485electron microscopy 1493histopathology 1484

sarcomatoid tumor differentialdiagnosis 1516–8

simian virus 40 1462–3small cell 1490solitary fibrous tumor of the


spread 1478–9squamoid change 1482staging 1469–71Stanton hypothesis 1467synchronous bilateral 1478TGF-b activity 1467thyroid transcription factor 1

negativity 1020tumor suppressor genes 1466,

1467vermiculite-associated 539–40well-differentiated papillary

1505–8clinical features 1505–6cytology 1507differential diagnosis 1507–8electron microscopy 1507pathology 1506, 1506–7prognosis 1508treatment 1508

Wilms’ tumor gene product1497–8

women 1458zeolite-associated 539see also sarcomatoid

mesotheliomamesothelioma, ovarian 1471–2mesothelioma, pericardial 1471mesothelioma, peritoneal 1458,

1471asbestos exposure 1461benign cystic 1522–3clinical presentation 1471deciduoid 1487desmoplastic 1485differential diagnosis 1485,

1527, 1528in women 1522–8

epithelioid 1479, 1480–2,1487–90

histology 1480–92immunohistochemistry 1527macroscopic pathology 1479–80microcystic 1487–8ovarian serous tumor


radiology 1471risk factors 1460–1sarcomatoid 1479, 1482–5Thorotrast exposure 1461well-differentiated papillary

1524mesothelioma, primary hepatic

1473mesothelioma, testicular 1473mesothelioma, tunica vaginalis

1472, 1472–3clinical presentation 1472treatment 1472well-differentiated papillary

1472mesothelioma cells 1467–8, 1474mesothelioma markers 1496–500mesothelium 13, 30–1

microvilli 31physiology 1409pneumothorax 1417structure 31surface proliferation 1415, 1417

metal-associated diseases 513,540–4

metal fume-induced disease546–50

metal-working fluids,hypersensitivitypneumonitis 459, 467

metaplastic bone 54metapneumovirus 117–18, 182metastases 118, 1375–401acinic cell carcinoma

differential diagnosis 1140adenoid cystic carcinoma

1135, 1394, 1382adrenal cortical carcinoma

1382alveolar soft-part sarcoma

1379ameloblastic fibrosarcoma

1394angiosarcoma 1383, 1392benign metastasizing

leiomyoma 1398breast carcinoma 1378, 1382,

1383, 1389, 1395calcification 393, 1378carcinosarcoma 1203cervical cancer 1398cervical lymph nodes 1394chondrosarcoma 1237, 1238–9,

1380chordoma 1387choriocarcinoma 1384, 1399clear cell carcinoma differential

diagnosis 1121clinical features 1376–7colorectal adenocarcinoma

1385, 1396, 1396colorectal carcinoma 1031,

1032, 1396CT imaging 1377dermatofibrosarcoma

protruberans 1392, 1400–1development 1375dormant micrometastases 1375endobronchial 1384–9endometrial carcinoma 1386,

1398endometrial sarcoma 1387stromal 1381, 1398

epithelialhemangioendotheliomadifferential diagnosis1266–7

gastrointestinal stromal tumor1396–7, 1396

gestational trophoblasticdisease 1398–9

giant cell sarcoma differentialdiagnosis 1197

glioblastoma multiforme 1387granulosa cell tumors of ovary

1398growth patterns 1377head and neck cancer 1393,

1394hemorrhage 1378hepatocellular carcinoma

1395–6hydatiform mole 1399immunohistochemistry 50incidence 1376interstitial spread 1391intra-alveolar spread 1391intra-pulmonary lymph nodes

1316intrathoracic nodal spread

1391–4leiomyosarcoma 1382lepidic spread 1390–1, 1396leukemia 1401liposarcoma 1384liver tumors 1395–6lung adenocarcinoma 1028,

1047, 1049lung cancer 970–1, 971, 1007,

1011lung site 1375–6lymphoepithelioma-like


lymphoma 1401malignant melanoma 1288,

1384, 1388, 1391, 1401diagnosis 1401differential diagnosis 1401intrathoracic lymph nodes1393

morphologic variability 1401treatment 1401

mechanical factors 1376meningioma 1394mesothelioma 1478–9differential diagnosis 1509–10

myofibroblastic tumor 1400–1nasopharyngeal carcinoma

1394neuroendocrine carcinoma

1399–400nodular 1377–81non-seminomatous germ cell

tumors 1399non-small cell lung cancer 971osseous osteogenic sarcoma

1235–6osteosarcoma 1389, 1400ovarian cancer 1391, 1397–8pancreatic adenocarcinoma

1385, 1391, 1396pancreatic carcinoma 1396parenchymal nodules 1377–81patterns 1377phyllodes tumor 1395, 1395placental site trophoblastic

tumor 1399

Index

1590






pleomorphic adenoma 1394pleomorphic carcinoma


pleuropulmonary blastoma122

prostate adenocarcinoma1399, 1400

pulmonary blastoma 1209pulmonary lymphangitic

carcinomatosis 1382–4radiography 1377renal cell carcinoma 1380,

1397, 1397rhabdomyosarcoma 123route of spread 1376salivary gland carcinoma 1394sarcoma 1400–1sclerosing hemangioma

869, 872seed and soil theory 1376sex cord stromal tumors 1398small cell lung carcinoma 1153soft tissue sarcoma 1400sources 1376spindle cell carcinoma

differential diagnosis 1194spindle cell sarcoma 864squamous cell carcinoma

1094, 1105synovial sarcoma 1283testicular non-seminomatous

germcell tumor 1381, 1399testicular seminoma 1399thyroid carcinoma 1395papillary 861, 872, 1378,1392

tongue carcinoma 1388tumor emboli 1389–90typical carcinoid tumor

differential diagnosis 1167unusual patterns of spread

1390–4urothelial carcinoma 1383,

1393uterine tumors 878–9uterine carcinoma 1384, 1398uterine leiomyoma 1398uterine leiomyosarcoma1398

methotrexate toxicity 590–1, 591drug-induced lymphoma 814,

1345methylphenidate abuse 600mica pneumoconiosis 538, 539Michaelis–Gutman bodies 412micro-aspiration, occult 466micro-CT scanning 48Micrococcus pneumonia 171, 178micronodular pneumocyte

hyperplasia (MNPH) 917see also multifocal

micronodularpneumocyte hyperplasia(MMPH)

microRNAs 908, 1104, 1105, 1466microscopic polyangiitis 729–31ANCA types 731autoantibodies 711clinical manifestations 730cytology 731differential diagnosis 731epidemiology 730genetics 730histopathology 730–1laboratory findings 731macroscopic pathology 730,

730, 731natural history 731pathogenesis 731polyarteritis nodosa differential

diagnosis 744–5prognosis 731radiographic findings 730

microsporidiosissee Encephalitozooncuniculi (microsporidiosis)

Microsporum canis, lung cancerrisk 957

microthrombi 58microvascular endothelial cells 30middle lobe syndrome 632–4, 634miliary nodular lesions 304see also Mycobacterium

tuberculosis (tuberculosis),miliary disease

Miller’s secondary lobules 11mineral dust inhalation 513,

531–2, 646, 647mineral fibers, non-asbestos

550, 550–3mineral pulmonary elastosis 58minimally invasive

adenocarcinoma (MIA)1044, 1053, 1058–9, 1066

mucinous 1068minute pulmonary

meningothelial-likenodules (MPMN) 1227–30

cell of origin 1228classification 1228clinical features 1227–8clinicopathologic correlation

1228differential diagnosis 1229electron microscopy 1228histopathology 1228immunohistochemistry 1228incidence 1227–8macroscopic pathology 1228molecular findings 1228natural history 1230nodules 1228prognosis 1230radiographic findings 1228reticulonodular infiltrates 1228

mitogen-activated protein kinase(MAPK) 1465

mitosis, pulmonary preinvasivedisease 903

mixed connective tissue disease827–8

alveolar hemorrhage 828autoantibodies 827clinical features 827diffuse alveolar damage 828interstitial lung disease 827pathology 827, 828pleural effusion 827–8pleurisy 827–8prognosis 828pulmonary hypertension 827

mixed dust fibrotic (MDF)lesions 532

mixed dust pneumoconiosis(MDP)/mixedpneumoconiosis 532, 532

MOC-31 1495molecular markers 43, 50–1Monge’s disease 686–8monoclonal antibodies (mAb)

592–3, 1021Monod sign, aspergilloma 232mononuclear cells, acute cellular

rejection morphologicalmimic 777

Moraxella catarrhalis 147, 178,171, 172

mordenite 538–9Morquio syndrome 422Mounier–Kuhn syndrome

71, 430mountain sickness, chronic

686–8mTOR signaling 864, 1258mucin(s) 18, 575, 612mucinous cystadenocarcinoma

1044–5, 1070, 1072, 1073mucinous cystadenoma 1072–3mucinous cystic neoplasms

1070–2, 1072mucociliary clearance, aging

effects 32mucociliary escalator, barrier to

bacterial infections 153mucoepidermoid carcinoma

1073, 1127–33adenoid cystic carcinoma

differential diagnosis 1133classification 1127clinical features 1127clinicopathological correlation

1131cytology 1129–30, 1131differential diagnosis 1132–3electron microscopy 1130genetics 1131high-grade 1129, 1131histopathology 1128–9, 1129,

1130immunohistochemistry 1130,

1132low-grade 1128–9, 1129, 1130macroscopic pathology 1127–8,

1128, 1129

mesothelioma differentialdiagnosis 1510

molecular findings 1131natural history/prognosis

1133radiographic findings 1127,

1128sclerosing hemangioma

differential diagnosis 872squamous cell carcinoma


treatment 1133mucopolysaccharidoses 421–3clinical manifestations 421–2electron microscopy 422, 422epidemiology 421genetics 421laboratory findings 423outcome 423pathology 422pathophysiology 423prognosis 423radiological findings 422treatments 423

Mucorales infections(mucormycosis) 239, 239

abscess 240clinical features 240culture 241diagnosis 241differential diagnosis 241endobronchial 240epidemiology 240organisms 239pathology 240prognosis 241pulmonary artery erosion 241treatment 241

mucosa-associated lymphoidtissue (MALT), marginalzone lymphoma1324–9

mucostasis, impaired carcinogenclearance 957

mucus cells see goblet cellsmucus gland adenoma (MGA)

1073, 1146–8, 1146clinical features 1146–7clinicopathologic correlation

1147definition 1146differential diagnosis 1147histopathology 1147immunohistochemistry 1147macroscopic pathology 1147mucoepidermoid carcinoma


natural history 1148prognosis 1148radiographic findings 1147

mucus retention cysts, tracheal 71Müllerian epithelial neoplasms

1473, 1524–5, 1528

Index

1591






multifocal micronodularpneumocyte hyperplasia(MMPH) 857–9, 858, 859

cell of origin 857classification 857clinical features 857–8clinicopathologic correlation 859definition 857differential diagnosis 859electron microscopy 859genetics 857, 859histopathology 858immunohistochemistry 858lymphangioleiomyomatosis

857–8macroscopic pathology 858molecular findings 859natural history 859prognosis 859radiographic findings 858

multiloculated peritonealinclusion cysts (MPIC)1522–3

multiple endocrine neoplasia(MEN), Carney’s triad1237

multiple myeloma 1340muscle dysfunction, COPD 623MUTYH-associated polyposis

960myasthenic syndrome 1153mycobacterial infections 206–23antigen presentation 213antigen processing modulation

212–13atypical (environmental) 206,

220, 222, 412, 458clinical signs 209diagnosis 207–8epidemiology 206–7management 223pathogenesis 213–14pathology 218–20radiological features 211

clinical management 222–3culture 207delayed-type hypersensitivity

reactions 213, 217differential diagnosis 221–2DNA sequences 208–9granular cell tumor differential

diagnosis 1242granulomas 213host genes 213host response 212lung cancer 956–7macrophage ingestion 212, 213microbiological diagnosis

207–9natural history 223pathogenesis 212–14pathology 214–22phagosomes 212–13prognosis 223serological tests 208

T cells 213TNFa 213virulence factors 212

Mycobacterium avium–intracellulare complex211, 218, 219–20,412, 458, 633–4

Mycobacterium immunogenum459

Mycobacterium kansasii 208Mycobacterium tuberculosis

(tuberculosis) 206advanced changes 214antibiotic resistance 208–9aspiration of necrotic

material 217bronchopneumonia 217, 217calcified hilar lymph node 216caseous necrosis 216cavitated pulmonary 217childhood epituberculosis 218clinical features 209clinical management 222–3clinicopathologic correlation

219–20delayed-type hypersensitivity

reactions 217diagnosis 207–9, 222diagnostic tests 208differential diagnosis 221–2diffuse pleural fibrosis 1447directly observed therapy

222–3drug-resistant 223drug side effects 223endobronchial 217, 219epidemiology 206gene sequence 209genome 209granulomatous inflammation

in lymph nodes drainingcancer 222

granulomatous pleural disease1423–4

hilar lymphadenopathy 212histopathology 214HIV coinfection 206, 209,

211, 223incidence 207inflammatory cell interactions

213laboratory investigations 209–10latent 209, 223lung cancer risk 956–7lymphadenopathy 212macroscopic pathology 214management 206mediastinal lymph node

disease 222miliary disease 209, 211, 211,

217, 218multi-drug resistant 206,

208–9, 223natural history 223necrotizing granuloma 215

nodal disease 211non-necrotizing granuloma 215pathogenesis 212–14pathological features 214–18pathology 214–22pleural disease 1423–4pleural effusion 210–11, 211,

217–18, 222, 1412pleural exudate 1412prevention 223primary pulmonary 216prognosis 223pulmonary syndromes 209radiological manifestations

210–11, 210, 211reactivated lesion 217secondary pneumothorax

1415–6secondary pulmonary

216–18, 219silicosis 529spindle cell pseudotumor

218, 219Takayasu arteritis association

748vasculitic process 215

Mycobacterium tuberculosiscomplex 206

Mycoplasma pneumoniaepneumonia 147, 176

mycosis fungoides 1346, 1346granulomatous variant 1346

mycotic infections see fungalinfections

myelodysplastic syndrome 1348myelofibrosis, chronic 1348myeloid leukemia 1347, 1347myeloid metaplasia 1348myeloid sarcoma 1347myeloperoxidase antibodies 711Myers–Kouwenaar body 310myoepithelial cells, epithelial–

myoepithelial carcinoma1136–7

myofibroblastic tumor 124–5,125, 126, 1278, 1400–1

myofibromatosis 125myxoid chondrosarcoma 1521myxoma 1273–4, 1522

N-acetylcholine receptor,nicotine addiction 964

N-cadherin 1499, 1503Napsin A 1023, 1066, 1103, 1190cytoplasmic staining 1023expression 1023, 1026

nasopharyngeal carcinoma,metastases 1386, 1394

NAT2 gene mutations, pleuralplaques 1440

Necator americanus 316necrotic nodule, solitary 314necrotizing sarcoid

granulomatosis 494,495, 1424

bronchiolitis obliterans 496diagnostic criteria 495differential diagnosis 498epidemiology 495laboratory findings 496natural history 498pathogenesis 497pathology 495, 496, 497prognosis 498radiographic findings 495vasculitis 495, 497, 498

Neisseria, pneumonia 178Neisseria meningitidis 172Neisseria mucosa 172botryomycosis 172

nematodes 221, 308–22neonatespulmonary vascular anatomy

666see also infants, perinatal lung

pathology, persistentpulmonary hypertensionof the newborn

nephrotic syndrome, pleuraleffusion 1411

nerve supply to lungs 12neural cell adhesion molecule

(NCAM) 1026, 1027,1164

neural neoplasms 1519neuroblastoma, metastatic 1519neuroendocrine antigens

1164–5neuroendocrine carcinoma,

metastases 1399–400neuroendocrine carcinoma of

lung, grades 1152neuroendocrine cell(s) 19–22, 20,

114–15bronchial 20bronchopneumonia 20, 21fetal lung 20neurosecretory vesicle 21–2, 22nodular aggregates 1172–3peritumoral proliferation

1177–8proliferative lesions 21, 22,

1152, 1172–8reactive proliferation 1172–3tumorlets 1172–3

neuroendocrine cell hyperplasiaof infancy 114–15, 641,1172

neuroendocrine hyperplasia,constrictive bronchiolitis641

neuroendocrine markers 1026,1027

neuroendocrine tumors 1151–78classification 1151–2clinical features 1152–3, 1154cytology 1157–64desmoplastic small round cell

tumor 1171diagnostic criteria 1152

Index

1592






differential diagnosis 1167–9electron microscopy 1166–7extrapulmonary metastatic

1168genetics 1166–7histopathology 1157–64immunohistochemistry 1164–5lung carcinoma with rhabdoid

phenotype 1171macroscopic pathology 1155natural history 1169paraganglioma 1167, 1171–2precursor lesions 1151primitive neuroectodermal

tumor 1170–1prognosis 1169radiographic findings 1153–7spectrum 1151, 1152see also carcinoid tumor, diffuse

idiopathic pulmonaryneuroendocrine cellhyperplasia (DIPNECH),large cell neuroendocrinecarcinoma (LCNEC),non-small cell carcinomas,with neuroendocrinedifferentiation,pleuropulmonaryblastoma, small cellcarcinoma, small cell lungcarcinoma (SCLC)

neuroepithelial bodies (NEBs)19, 20, 20, 910

neurogenic tumors 1239neuron-specific enolase (NSE)

21, 1027, 1164–5neurosecretory vesicle, pulmonary

neuroendocrine cells21–2, 22

neutropenia, Aspergillus infection229

neutrophil(s)acute lung injury/ARDS 346,

351–3, 354ANCA interactions 711, 729asthma 566Behçet syndrome 751COPD 611emphysema 620function 153usual interstitial pneumonia/

idiopathic pulmonaryfibrosis 373

viral infections 185Wegener granulomatosis 728

neutrophilic capillaritis 450nicotineactivation 964addiction 964clearance 950–1

Niemann–Pick cells 413, 414–15,416–17

Niemann–Pick disease, typeA and B 414–16, 414

clinical manifestations 414

cytology 415differential diagnosis 416electron microscopy 415, 415epidemiology 414genetics 414laboratory findings 415natural history 416pathology 414–15, 415pathophysiology 416prognosis 416radiological findings 414

Niemann–Pick disease, type C416–17

clinical manifestations 416epidemiology 416genetics 416laboratory findings 417pathology 416–17pathophysiology 417prognosis 417radiological findings 416treatment 417

nitric oxide (NO) 113, 1409nitric oxide synthase, inducible

(iNOS) 1409nitrofurantoin toxicity 592nitrogen oxide, lung

adenocarcinomaassociation 952

nitrosamines 891NK/T-cell lymphoma, nasal type

1335NKX2–1 amplification 1064Nocardia asteroides 166, 167, 177abscess 166granulomatous reaction 167in multinucleated giant cell 167

nodular density, wedge biopsy 44nodular lymphoid hyperplasia

825, 1321–2, 1322benign pulmonary lymphoid

proliferations 1318clinical features 1321differential diagnosis 1321–2pathology 1321

non-Hodgkin lymphoma 825non-small cell carcinomaswith giant cells 1197with neuroendocrine

differentiation 1170non-small cell lung cancer

949, 978, 1285age 948metastatic sites 971

nonspecific interstitialpneumonia (NSIP) 114,201, 367, 374–9, 445,463, 524

alveolar cell hyperplasia 916asbestosis differential

diagnosis 522associated conditions 376cellular 376–7, 378, 588classification 376clinical presentation 375

connective tissue diseasepattern of involvement804

dermatomyositis 826desquamative interstitial


differential diagnosis 377–8drug-induced lung disease

587–8fibrotic 377, 378genetics 368histological features 377histological pattern 375HIV infection 266hypersensivity pneumonitis

451, 455lymphoid interstitial


lymphoid proliferation 1318mixed cellular/fibrotic 376natural history 379pathogenesis 379pathology 375–7, 376polymyositis 826, 826prognosis 379radiological findings 375,

375, 444respiratory bronchiolitis/

RB-ILD differentialdiagnosis 387

rheumatoid arthritis 809, 810scleroderma 821–2, 821Sjögren syndrome 823, 824systemic lupus erythematosus

818undifferentiated connective

tissue disease 829Noonan syndrome,

congenital pulmonarylymphangiectasis 87

NPC gene mutations 416nuclear factor-kB (NFkB) 153,

906, 1465, 1466nucleic acid amplification tests

(NAATS) 208nucleophosmin 904nuisance dusts 545–6nylon flock worker’s lung 551, 553

obesity, lung cancer risk 960obliterative bronchiolitis 186,

640–3, 792, 793bronchiectasis 794clinical features 811constrictive 553, 641–3differential diagnosis 792etiology 812histologic features 792, 794, 811lung-resident mesenchymal

stromal cells 4lung transplantation 768, 790–2necrotizing sarcoid

granulomatosis 496

occupational 553patterns 793prognosis 812proliferative 640rheumatoid arthritis 811–12,

812treatment 792Wegener granulomatosis 726–7

obstructive sleep apnea, Marfansyndrome 428

occult micro-aspiration 466occupational lung disease 512–53aluminum lung disease 549asbestos 512–28barium pneumoconiosis 546benign pneumoconiosis 545–6beryllium exposure 540–1cadmium pneumonitis 550carbon fibers 551–3coal/coalmine dust 532–6dental technician’s

pneumoconiosis 545economic cost 512food flavorer’s lung disease

553hard metal lung disease 541–3lung cancer occupational

factors 952–3, 954, 955man-made fibers 550–1mercury pneumonitis 550metal-associated diseases

513, 540–4metal fume-induced disease

546–50mineral dust inhalation 513,

531–2mixed dust pneumoconiosis/

mixed pneumoconiosis532, 532

non-asbestos mineral fibers550–3

non-asbestos silicatoses 536–40nuisance dusts 545–6nylon flock worker’s lung 551pathologic response to agents

512popcorn worker’s lung disease

553rare earth pneumoconiosis

544–5silicon carbide

pneumoconiosis 544silicotic lung disease 528–32stannosis 546titanium pneumoconiosis

545–6welder’s pneumoconiosis

547–9Odland bodies, squamous cell

carcinoma 1103oligohydramnios, pulmonary

hypoplasia 91–2, 93Onchocerca volvulus

(onchocerciasis) 308, 322oncocytic metaplasia 54

Index

1593






oncocytoma 1147, 1148, 1148oncogenes 907open lung biopsy, lung

transplantation 770organ transplantationamebiasis 294CMV antigen 196CMV risk 195immunosuppressive drugs 1348Kaposi’s sarcoma 1268lung cancer risk with

immunosuppression 956microsporidiosis 304see also lung transplantation,

post-transplantlymphoproliferativedisease (PTLD)

organic toxic dust syndrome439, 443, 491

organization of lungs 7–13blood supply 10–11, 28–30fissures 8lobes 8lymphatics/lymph nodes 11–12nerve supply 12pleura 12–13, 30–1size 8structural anomalies 8weight 8

organizing pneumonia (OP)connective tissue disease pattern

of involvement 804dermatomyositis 826, 826drug-induced lung disease 588hypersensitivity pneumonia

differential diagnosis 463inflammatory bowel disease

833–4lung-resident mesenchymal

stromal cells 4nonspecific interstitial

pneumonia pattern 378pathology 588polymyositis 826progression to interstitial

fibrosis 371rheumatoid arthritis 809, 810Sjögren syndrome 824steroid therapy 809systemic lupus erythematosus

819see also acute fibrinous

organizing pneumonia(AFOP), cryptogenicorganizing pneumonia(COP)

Orientia tsutsugamushipneumonia 179

ornithosis 148, 175oropharynx, bacterial

colonization 153osseous osteogenic sarcoma

metastases 1235–6ossifying periostosis 973osteogenic sarcoma 1521

osteopontin 368, 1529–30osteoporosis, COPD 622osteosarcomametastatic 1389, 1400pulmonary 1236see also primary pulmonary

osteogenic sarcomaovarian cancer, metastases 1391,

1397–8ovarian serous tumors 1527, 1528overinflation, biopsy artefact 51oxidative stresslung cancer 958preterm infants 108–9

p-glycoprotein(s) 1235p14arf 904p16/CDKN2A deletion in

mesothelioma 1529p16 gene function loss 905,

907, 909p16 protein 904–5, 923, 1027p21 protein 922p27 protein 925p53 proteinatypical adenomatous

hyperplasia 922–3function 903pulmonary preinvasive disease

903–4squamous dysplasia/

carcinoma-in-situ 904p53 tumor suppressor geneatypical adenomatous

hyperplasia 923BaP diol epoxide 951carcinosarcoma 1204gene mutations 903–4large cell carcinoma 1115lung cancer 951mesothelial hyperplasia

1453–4mesothelioma 1504pleomorphic carcinoma 1190pulmonary blastoma 1213squamous cell carcinoma

1103p63 protein 904atypical adenomatous

hyperplasia 923immunohistochemistry 1027lung adenocarcinoma 1061pleomorphic carcinoma 1190squamous cell carcinoma


p170 glycoprotein 1505pachypleuritis see diffuse pleural

fibrosisPagetoid reticulosis 1346pAKT 905Pancoast tumor 970, 970, 976,

1094–5pancreatic adenocarcinoma,

metastases 1385,1391, 1396

pancreatitis, autoimmune 1420panniculitis, COPD 622Panton–Valentine Leucocidin

(PVL) toxin 148papillary adenoma 859–62, 860atypical adenomatous



861cytology 860–1, 860, 861definition 859differential diagnosis 861electron microscopy 861etiology 859histopathology 860immunohistochemistry 861lung adenocarcinoma


macroscopic pathology 860molecular findings 861natural history 861–2prognosis 861–2radiographic findings 860sclerosing hemangioma

differential diagnosis 872papillary carcinoid tumor 861papillary cystadenoma 861papillary predominant

adenocarcinoma 1054–5papillary thyroid carcinoma 1377metastatic 861, 872, 1378, 1392

Paracoccidioides brasiliensis(South Americanblastomycosis) 262–4, 262

acute disease 263antigen detection tests 264blastomycosis differential

diagnosis 257clinical features 263culture 263diagnosis 263–4differential diagnosis 264dissemination 263enzyme immunoassay 264epidemiology 262–3immunodiffusion test 263–4inflammatory response 263mucocutaneous lymphangitis

263organism 262pathology 263progressive pulmonary

disease 263serological tests 263–4subacute disease 263treatment 264

paraganglioma 1167, 1171,1171–2, 1249

Paragonimus (paragonimiasis)326–8

clinical features 326–7diagnosis 327–8eggs 327epidemiology 326organism 326, 326, 327pathology 327, 327radiological features 326–7

parainfluenza viruses 184, 192,192

bronchiolitis 192diagnosis 192pathology 192

paraneoplastic cerebellardegeneration 974

paraneoplastic pemphigus 1278paraneoplastic syndromescentral nervous system 973–4cutaneous 974dermatomyositis 827, 974–5hypoglycemia 1431large cell carcinoma 1114lung carcinoma 971–5polymyositis 827retinopathy 974small cell lung carcinoma 1153solitary fibrous tumor of the

pleura 1431squamous cell carcinoma 1094

paraquat toxicity 598, 598parasitic disease 288–335, 736, 1425see also named organisms and

taxonomic groupsparathyroid hormone (PTH) 971parathyroid hormone-related

peptide (PTHrP) 971parenchymal nodules, metastases

1377–81particle mass (PM) 952Pasteurella multocida

pneumonia 173, 178pathogen-associated molecular

patterns (PAMPs)229–30

pattern recognition receptors153, 229–30, 1410

PE-10 1026, 1026pemetrexed, contraindication in

squamous cell carcinoma1104

Penicillium marneffei(penicilliosis marneffei)228, 249

pentastomiasis 334, 335Peptostreptococcus 159, 175pergolide mesylate 1445peribronchial interstitial

fibrosis 391peribronchiolar metaplasia 391,

646–8, 648, 917, 918atypical adenomatous


hypersensitivity pneumonitisdifferential diagnosis 464

pathophysiology 646–8

Index

1594






prognosis 648treatment 648

pericytes 30perinatal lung pathology 102–13air leaks 109–11embolism 112hyaline membrane disease

103–6meconium aspirate syndrome

112–13persistent pulmonary

hypertension of thenewborn 113

pleuropulmonary blastoma119–23

pneumothorax 109–11pulmonary hemorrhage 111–12pulmonary interstitial

emphysema 109–11respiratory distress syndrome

103–6surfactant metabolic

dysfunction 106thrombosis 112ventilation 109, 111Wilson-Mikity syndrome 109see also bronchopulmonary

dysplasiaperipheral lung

adenocarcinogenesis921–2, 927–8, 929

peripheral lung cyst 81peripheral lung epithelium,

carcinogenesis 909–10peripheral nerve sheath tumors

1518–9peritoneal angiomatoid tumor

1524peritoneal carcinoma, primary

1527peritoneal dialysis, pleural

effusion 1411peritoneal inclusion cysts

1522–3, 1524peritoneal serous carcinomahigh-grade 1526–7low-grade 1526

peritoneal serous lesions 1524–5peritoneal serous tumors 1526,

1527perivascular epithelioid cell

neoplasms (PEComas)1256–8

persistent pulmonaryhypertension of thenewborn 113

nitric oxide 113nitric oxide therapy 113vascular resistance 113

Peutz–Jeghers syndrome,STK11/LKB1 gene 1063

Pfeiffer syndrome, trachealstenosis 68

PGP9.5 1026phaeohyphomycoses 277–8, 277

diagnosis 278etiology 277–8organisms 277subcutaneous 277treatment 278

phagosomes, mycobacterialinfections 212–13

phenethylamine-derived drugabuse 600

phosphatidylcholine 25–6phosphatidylcholine 3-kinase

(P13-K/AKT) pathway1466

phospholipase A2 680photography of large specimens 47phyllodes tumor, metastases

1395, 1395physical activity, lung cancer risk

960phytosterols, lung cancer 960Pierre–Marry–Bamberger

syndromesee hypertrophicpulmonaryosteoarthropathy(HPOA)

pink puffers, COPD 607placental site trophoblastic

tumor (PSTT),metastases 1399

placental transmogrification 625,626, 626–7, 627

plague 179plasma cell disorders 1337plasmacytic hyperplasia (PH)

787plasmacytoma 1342clinical features 1341differential diagnosis 1341immunohistochemistry 1341pathology 1341, 1341treatment 1341

Plasmodium falciparum 297, 297,300

Plasmodium ovale 297Plasmodium vivax 297plastic bronchitis 635, 635platelet-derived growth factor

(PDGF) 1466–7platelet-derived growth factor

receptor (PDGFR)1433–5

platelets, acute lung injury 354pleomorphic adenoma 1141,

1141–4cell of origin 1141classification 1141clinical features 1141clinicopathologic correlation

1143cytology 1143, 1143differential diagnosis 1143–4electron microscopy 1143genetics 1141histopathology 1141–2, 1142

immunohistochemistry 1143macroscopic pathology 1141metastases 1394molecular findings 1143natural history 1144, 1195pneumocytic

adenomyoepitheliomadifferential diagnosis1145

prognosis 1144, 1195radiographic findings 1141

pleomorphic carcinoma 1187–95,1188

adenocarcinoma 1188b-hCG expression 1190clinical findings 1187CYP1A1 1191–2cytokeratins 1188–9cytology 1188definition 1187differential diagnosis 1192–5EGFR gene mutation 1190–1electron microscopy 1190, 1195EML4-ALK fusion gene 1191epithelial membrane antigen

1188–9histopathology 1187–8, 1189,

1190, 1191immunohistochemistry

1188–90, 1195KRAS gene mutations 1190macroscopic pathology 1187metaplastic 1196molecular findings 1190–2p53 gene mutations 1190p63 gene expression 1190prognosis 1195radiographic findings 1187,

1187surfactant protein A 1189survival 1195thyroid transcription factor 1

1189pleomorphic sarcoma 1197, 1275pleura 30–1asbestos fibers route of entry

1440basement membrane 31biopsy 49black spots 13, 31connective tissue 31elastic layer 31, 31entrapped 59, 60foreign material 1417Kampmeier focus 32mesothelial layer 30–1organization of lung 12–13primary squamous carcinoma

1509reactive eosinophilic vascular

infiltration 1418sarcoidosis 482, 1424

pleural disease 1408–9adenocarcinoma 1494benign 1503–5

asbestos-related 1436–54calcifying fibrous tumor

1429–30connective tissue diseases

1425–6drug-induced lung disease

589–90, 1412, 1426–7endometriosis 1427–8granulomatous 1423–5parasitic 1425rheumatoid arthritis 807, 1425,

1426systemic lupus erythematosus

1425–6Wegener’s granulomatosis

1424, 1424–5see also named diseases and

conditionspleural effusion 1410–4aspergillosis 1412atelectasis 1411–2benign asbestos-associated

1436–7, 1443–4clinical manifestations1443–4

definition 1443differential diagnosis 1444epidemiology 1443etiology 1443lag period from asbestosexposure 1443

pathology 1444congestive heart failure

1410–1eosinophilic 1412, 1414exudates 1410, 1412hepatic cirrhosis 1411infective 1412lactate dehydrogenase 1410lung cancer 977metastatic breast carcinoma

1395mixed connective tissue

disease 827–8natural history 1444nephrotic syndrome 1411pathophysiology 1413–4peritoneal dialysis 1411physiology 1410pleural fluid increase 1410prognosis 1444pulmonary hypoplasia 93radiology 1412–3size 1410systemic lupus erythematosus

1425–6transudates 1410–2treatment 1414tuberculosis 210–11, 211,

217–18, 222, 1412urinothorax 1411

pleural fibromatosis 1443pleural fibrosis 1419–36, 1445,

1446, 1447–8see also diffuse pleural fibrosis

Index

1595






pleural fluid 1408–9cell content 1409clinical features 1410composition control 1409distribution 1408exchange 1408lymphatic drainage 1408–9mesothelial cells 1408, 1409–10pleural effusion 1410processing 41–2removal 1408–9Starling forces 1409volume 1409volume control 1409

pleural granulomas, calcified 1430pleural lymphoma 1354pleural myxoma 1521pleural patches 13, 31pleural plaques 1436–43apical cap differential

diagnosis 1423asbestos exposure level 1437calcifying fibrous tumor

of the pleura differentialdiagnosis 1430

causes 1439clinical features 1440coronary artery disease

association 1436–7differential diagnosis 1442–3epidemiology 1437–8etiology 1438–9factors affecting production 1437fiber type 1437folded lung differential

diagnosis 1451genetics 1440histopathology 1441–2incidence 1438lag period from asbestos

exposure 1440macroscopic pathology 1441malignancy risk 1443mesothelioma 1443, 1476differential diagnosis 1442–3

non-asbestos causes 1439–40radiography 1437, 1440–1smoking 1439–40

pleural sarcoidosis 482pleural space 1408pleural surface pressure 1408pleural tumors 50adenomatoid 1512melanoma 1513psammoma bodies 1510thymoma 1511, 1512

pleurisy 1412, 1413, 1425Aspergillus 1413fibrosing 1485mixed connective tissue

disease 827–8nonspecific 1420organizing 1487systemic lupus erythematosus

1425

pleuritisbilateral cryptogenic fibrosing

1445chronic fibrinous 1430familial cryptogenic fibrosing

1445idiopathic 1419–20idiopathic lymphocytic 1449rheumatoid arthritis 806systemic lupus erythematosus

815, 1423pleuroparenchymal fibroelastosis

1448pleuropulmonary blastoma

118–23, 1215clinical features 119differential diagnosis 123genetics 122–3histopathology 119–21macroscopic pathology 119pathogenesis 121prognosis 122pulmonary hamartoma


radiographic abnormalities 119treatment 121Types I–III 120, 121, 121,

122, 123see also pulmonary blastoma

pleuropulmonary fibroelastosis391, 1423, 1448

plexogenic arteriopathy 673–9,676

arterial intimal fibrosis 674–5arteritis 678associated conditions 674cardiac defects 697congenital heart disease

673, 697differential diagnosis 677–8epidemiology 674focal arteritis 676histopathology 674–7, 677, 678medial hyperplasia 674necrotizing arteritis 676plexiform lesion 676risk factors 674Schistosoma infection 684systemic lupus erythematosus

817treatment 678–9vascular lesions 674VEGF1 677

pneumoconiosisbarium 546benign 545–6dental technician’s 545endogenous 58, 693kaolin 538mica 538non-asbestos silicatoses 536–8radiographic classification 521rare earth 544–5talc 536–8

titanium 545–6tungsten carbide 541–3welder’s 547–9see also coal workers’

pneumoconiosis(CWP), mixed dustpneumoconiosis (MDP)/mixed pneumoconiosis,rheumatoidpneumoconiosis (RP)

Pneumocystis jirovecii pneumonia(PCP) 268–72, 271

atypical 269cavitary masses 271classical (typical) 269clinical features 269coin lesions 271diagnosis 269–72differential diagnosis 272epidemiology 268–9extra-pulmonary 269b-D-glucan detection 272granulomatous pneumonitis 466histology 270hypersensitivity pneumonitis


immunocompromised patients268–9

interstitial disease 269localized nodular 269lung transplantation 785molecular diagnostics 272necrotizing granulomas 271organism 268pathology 269prognosis 272pulmonary alveolar

proteinosis differentialdiagnosis 397

secondary pneumothorax1415–6

serological tests 271systemic dissemination 271transmission 268treatment 272

pneumocytesdevelopment 4type I 24–5, 25, 355type II 24, 25–6, 25, 26, 355acute lung injury/ARDS346–7

function 25–6hyaline membrane disease105, 106

hyperplasia 348, 380, 916,917

Mallory’s hyaline-likematerial 56, 56

microvilli 25reactive hyperplasia 377

type III (see brush cells)pneumocytic

adenomyoepithelioma1144–5, 1145

clinicopathologic correlation1145

differential diagnosis 1145electron microscopy 1145histopathology 1144immunohistochemistry 1144–5macroscopic pathology 1144,

1144natural history 1145prognosis 1145

pneumonectomy 45–7, 1530pneumoniapleural exudate 1412see also interstitial lung

disease, named typesof pneumonias

pneumonia, bacterialanaerobic bacteria 148, 155–75aspiration 146, 148, 149atypical 147–8, 149bacterial 115–16, 146botryomycosis 176causes 156cavitation of lung 151Chlamydiaceae 175classification 146clinical manifestations 148–9community-acquired 146, 147,

147, 148diagnosis 151, 155early-onset 115–16Enterobacteriaceae 175–6epidemiology 146–8etiology 146–8group B streptococcal

infection 115–16histologic features of bacteria

155host defense 152–4in the immunocompromised

146incidense 146infection events 149–50inflammatory nodules 151inhalation of contaminated

air 149late-onset 116lung shadowing 151management 154microbiological workup 154–5Mycoplasma 176natural history 154nosocomial 146, 148organizing 150pathogenesis 152–4patterns of injury 149pneumococcal 147, 177prognosis 154pseudolipoid 52–3radiologic manifestations

151–2Rickettsiales 179routes of injury 149–51Salmonella 179staining of bacteria 155

Index

1596






staphylococcal 152, 176Streptococcaceae 176–7see also interstitial pneumonia

pneumonia, fungal 147, 226pneumonia, viral 118, 147, 182–3adenovirus 118, 182–3herpes 117influenza 189measles 183, 193respiratory syncytial virus

118, 191pneumonia hypersensitivity 371pneumonitiscadmium 550CMV 184granulomatous 465, 466mercury 550Mycobacterium avium–

intracellulare complexassociated 219–20

pneumothorax 109–11, 1414–9age factors 1415blebs/bullae 1415, 1418catamenial 1416clinical features 1416–7cysts 1418differential diagnosis 1419eosinophilic infiltration 1418epidemiology 1414, 1414–5genetics 1415, 1416iatrogenic 1416lymphangioleiomyomatosis

1254, 1255Marfan syndrome 428mesothelial proliferation 1417mesothelioma 1469pathology 1417, 1417–8pathophysiology 1418–9primary spontaneous 1414,

1415rheumatoid arthritis 1425risk factors 1415secondary 1414, 1415–6tension 110

podoplanin, mesotheliomadiagnosis 1499

poly-ADP-ribose polymerase(PARP) 1465

polyalveolar lobe 85polyarteritis nodosa 700, 743–5clinical manifestations 744definition 743diagnostic criteria 744differential diagnosis 744–5electron microscopy 744epidemiology 743–4histopathology 744, 745immunohistochemistry 744infection association 743–4laboratory findings 744macroscopic findings 744, 745morphologic stages 744natural history 745pathogenesis 744prognosis 745

pulmonary involvement 743radiographic findings 744

polycyclic aromatichydrocarbons (PAH) 891

polyhydramnios, congenitalperibronchialmyofibroblastic tumor 124

polymyalgia rheumatica,genetics 746

polymyositis 825–7acute fibrinous organizing

pneumonia 827anti-Jo-1 826aspiration pneumonia 827diffuse alveolar damage 826interstitial lung disease 826lung carcinoma 827, 958nonspecific interstitial

pneumonia pattern826, 826

organizing pneumoniapattern 826

paraneoplastic syndrome974–5

pathology 825prognosis 826usual interstitial pneumonia

pattern 826polysplenia syndrome,

arteriovenousmalformations 1261

Pompe disease 425, 425–6clinical features 425electron microscopy 425genetics 425pathology 425prognosis 425–6radiological findings 425treatment 426

popcorn worker’s lung disease 553pores of Kohn 26, 26portal hypertension, plexogenic

arteriopathy 668–70posaconazole, Fusarium

infection treatment 244post-cardiac injury syndrome

1420post-obstructive vasculopathy,

bronchial arteries 665post-transplant

lymphoproliferativedisease (PTLD) 768–9,1345

acute cellular rejectionmorphological mimic 778

classical Hodgkin lymphomatype 788–9

classification 787definition 786diagnosis 787early lesions 787EBV-associated 199, 778,

786–9, 1345immunosuppressive drugs

1348

infectious mononucleosis-like787

monomorphic 788patterns 788polymorphous 787–8risk factors 786treatment 786

Potter’s syndrome, pulmonaryhypoplasia 92

pregnancy, sarcoidosis 490preterm infants 107, 108–9primary ciliary dyskinesia 95–7cilial orientation 96ciliary beat assessment 96diagnosis 95genetics 95investigations 95–6lung disease 96–7ultrastructural studies 96

primary effusion 1355primary effusion lymphoma

1268, 1356clinical features 1355differential diagnosis 1355immunohistochemistry 1355molecular pathology 1355pathology 1355

primary pulmonaryangiosarcoma 1272–5

cell of origin 1272clinical features 1272clinicopathologic correlation

1273differential diagnosis 1273electron microscopy 1273histopathology 1273immunohistochemistry 1273Kaposi’s sarcoma differential

diagnosis 1272macroscopic pathology 1273molecular findings 1273natural history 1273prognosis 1273radiographic findings 1272–3treatment 1273

primary pulmonarychondrosarcoma 1238,1238–9

differential diagnosis 1238–9pathology 1238prognosis 1239

primary pulmonary glomustumors 1167, 1247–9


1249cytology 1248differential diagnosis 1249electron microscopy 1249histopathology 1248immunohistochemistry

1248–9macroscopic pathology 1248

malignant 1248molecular findings 1249radiographic findings 1248succinated dehydrogenase

missense mutation 1249primary pulmonary leiomyoma/

leiomyosarcoma 1242–3primary pulmonary lipoma

1231–3age factors 1231cell of origin 1231classification 1231clinical features 1231clinopathologic correlation

1232cytology 1232differential diagnosis 1233electron microscopy 1232genetics 1231histopathology 1231immunohistochemistry 1232macroscopic pathology 1231molecular findings 1232natural history 1233prognosis 1233radiographic features 1231

primary pulmonary liposarcoma1233–4, 1521


1234dedifferentiated 1233differential diagnosis 1234electron microscopy 1233genetics 1233histopathology 1233immunohistochemistry 1233macroscopic pathology 1233molecular findings 1234myxoid 1233natural history 1234pleomorphic 1233prognosis 1234radiographic findings 1233

primary pulmonary malignantmelanoma 1286–8, 1401


1288cytology 1287differential diagnosis 1288electron microscopy 1287genetics 1286histopathology 1287immunohistochemistry 1287macroscopic pathology 1287molecular findings 1287natural history 1288pagetoid spread 1287prognosis 1288radiographic findings 1287

Index

1597






primary pulmonary meningioma1230–1

cell of origin 1230classification 1230clinical features 1230clinicopathological correlation

1230cytology 1230differential diagnosis 1230–1electron microscopy 1230histopathology 1230immunohistochemistry 1230macroscopic pathology 1230minute pulmonary

meningothelial-likenodule differentialdiagnosis 1229

natural history 1231prognosis 1231radiographic findings 1230

primary pulmonary osteogenicsarcoma 1235–6

classification 1235clinical features 1235clinicopathologic correlation

1235CXCR4 chemokine receptor

1235differential diagnosis 1235–6histopathology 1235immunohistochemistry 1235macroscopic pathology 1235molecular findings 1235natural history 1236P-glycoprotein 1235prognosis 1236radiographic findings 1235

primitive neuroectodermaltumor (PNET) 1170–1,1171, 1249, 1519–20

probe method of dissection 49progenitor cells, lung

development 7progressive massive fibrosis

(PMF) 533, 534, 535, 535coal workers’ pneumoconiosis

535complicated 534differential diagnosis 535–6rounded atelectasis 1451talcosis 537

prostate adenocarcinoma,metastases 1399, 1400

protein gene product 9.5(PGP 9.5) 21, 1164–5

proteinase 3 622, 711Proteus, pneumonia 116Proteus mirabilis pneumonia 175protozoal infections 288, 289–307amebiasis 291–5babesiosis 300Balantidium coli 307cryptosporidiosis 305–7Cyclospora 307leishmaniasis 289–91

Lophomonas 307malaria 297–300microsporidiosis 304–5toxoplasmosis 301–4Trichomonas 307trypanosomiasis 295–7

psammoma bodies 1510, 1526Pseudallescheria boydii 241, 242pseudoangiosarcomatous

carcinoma 1193pseudoepitheliomatous

adenocarcinoma 1509pseudolymphoma see nodular

lymphoid hyperplasiapseudomesotheliomatous

adenocarcinoma 1065Pseudomonas 116, 116Pseudomonas aeruginosa 162, 176cystic fibrosis 99, 100

Pseudomonas cepaciasee Burkholderia cepacia

Pseudomonas fluorescens 459pseudopneumoconiosis 693psittacosis 148, 175pulmonary adenocarcinogenesis

924see also peripheral lung

adenocarcinogenesispulmonary agenesis 74–5bilateral 74, 75lobar 75unilateral 74–5

pulmonary alveolar microlithiasis394, 394, 395

diagnosis 394genetics 394natural history 394pathology 394prognosis 394radiological findings 394

pulmonary alveolar proteinosis(PAP) 394–7, 396, 397

childhood/infant 395clinical course 397clinical features 395–6diagnosis 396, 397differential diagnosis 397drug-induced lung disease 589etiology 394–5GM-CSF 395, 397idiopathic 395opportunistic infections 396–7pathology 396–7prognosis 397radiological findings 396secondary 395type II 397

pulmonary arterial tension,HIV-related 201–2

pulmonary arterial trunk 11, 32pulmonary arteries 28–9adventitia 28, 29changes 666, 689

aging 32anatomy 663–4

concentric laminar intimalfibrosis 675

development 4elastic 28–9intima/media 28medial hyperplasia 675muscular 28, 29, 29newly formed muscular media

675occlusion with

thromboembolism/thrombosis 680

organization 11stenosis 72supernumerary arterial

branches 663, 664–5, 666walls 28

pulmonary arterioles 28, 29–30,663–4

pulmonary arteriovenousmalformation 1262

pulmonary artery aneurysms,Behçet syndrome 831

pulmonary artery sarcoma1278–81

cell of origin 1279classification 1279clinical features 1279clinopathologic correlation

1281differential diagnosis 1281electron microscopy 1279genetics 1279histopathology 1279immunohistochemistry 1279macroscopic pathology 1279molecular findings 1281natural history 1281prognosis 1281radiographic findings 1279treatment 1281

pulmonary blastoma 1170, 1187,1205–16

age factors 1205blastomatoid carcinosarcoma

differential diagnosis1203, 1214–5

carcinosarcoma differentialdiagnosis 1199–200, 1204

b-catenin gene mutation1212–3

cell of origin 1207clinical features 1203, 1206,

1207cytology 1210, 1212, 1213definition 1206–7differential diagnosis 1211,

1214–5electron microscopy 1211–2epidemiology 1207epithelial cells 1210glandular cells 1209histopathology 1209, 1209,

1210historical overview 1205–6

immunohistochemistry1210–1, 1214, 1215

macroscopic pathology 1208–9,1208

melanocytic differentiation 1213mesenchymal cells 1210, 1211mesenchymal component

1209metastases 1209molecular findings 1212–3morular formation 1209,

1210–1, 1211–2natural history 1215–6p53 gene mutations 1213pathological features 1203,

1206pleomorphic adenoma


prognosis 1215–6radiographic findings 1207,

1208smoking 1207stromal cells 1212studies 1205–6treatment 1216Wnt signaling pathway

activation 1212see also pleuropulmonary

blastomapulmonary calcification 392–3dystrophic 392metastatic 392–3pathology 393treatment 393

pulmonary capillaries 29–30, 665pulmonary capillary

hemangiomatosis (PCH)695

associated conditions 692capillary proliferation 693clinical features 691differential diagnosis 693epidemiology 691hemosiderin deposition 693histopathology 693intimal fibrosis 693medial hyperplasia 693pulmonary hypertension

691–3radiologic features 692–3treatment 691vascular lesions 696

pulmonary chondroma 1237–8Carney’s triad 1237cell of origin 1237classification 1237clinical features 1237differential diagnosis 1237genetics 1237histopathology 1237macroscopic pathology 1237natural history 1238prognosis 1238radiographic findings 1237

Index

1598






pulmonary circulatory system10–11, 663, 665–6

pulmonary cystic disease 75–82acinar dysplasia 82age 76bronchogenic cysts 77–8congenital adenomatoid

malformations 78–82diagnosis 76–7diagnostic criteria 76spectrum 76

pulmonary diffusion, alveolarvolume relationship 7

pulmonary ectopic deciduosis 879pulmonary edema 568–9pulmonary embolism 153, 684chronic thromboembolic

pulmonary hypertensionrisk 679

foreign body 686malignancy 684non-thrombotic causes 684–6pulmonary artery sarcoma

1281radiological findings 667tumor emboli 1389, 1390

pulmonary eosinophiliasyndromes 308, 309

pulmonary epithelialhemangioendothelioma(EHE) 1263–8

pulmonary fibrosis 349coal dust-induced 532–3diffuse interstitial 536Hermansky–Pudlak syndrome

419, 421idiopathic 608mixed dust pneumoconiosis

532paraquat toxicity 598pediatric lung transplantation

768see also progressive massive

fibrosis (PMF)pulmonary glial nodules 90pulmonary glomangiosarcoma

1248, 1249pulmonary hamartoma 1224–7age factors 1224angiomyolipoma differential

diagnosis 1256cell of origin 1224classification 1224clinical features 1224clinicopathologic correlation

1227cytology 1225, 1227diagnosis 1225differential diagnosis 1227electron microscopy 1225endobronchial 1224–5genetics 1224histopathology 1225macroscopic pathology

1224–5, 1226

natural history 1227pleomorphic adenoma


primary pulmonary lipomadifferential diagnosis1233

prognosis 1227pulmonary chondroma


radiographic features 1224,1225

pulmonary hemorrhage 111–12ANCA 742causes 742differential diagnosis 742–3drug-induced lung disease

589, 742prognosis 743pulmonary endometriosis

differential diagnosis 879pulmonary–renal syndromes

742treatment 743

pulmonary hemorrhagesyndromes 740–5, 740

antiphospholipid syndrome741, 742

pulmonary hemosiderosis,idiopathic 742

pulmonary hyalinizinggranuloma (PHG)872–4, 873

associated diseases 872–3clinical features 872–3clinicopathological correlation

874differential diagnosis 874electron microscopy 874epidemiology 872–3etiology 872–3histopathology 873–4, 874macroscopic pathology 873,

873natural history 874pathogenesis 874prognosis 874radiologic features 873serologic associations 873

pulmonary hyperplasia 86–7, 87,90, 1242

pulmonary hypertension 662–3alveolar capillary dysplasia 89animal models 668antiphospholipid syndrome 820appetite suppressants 670associated conditions 661BMPR2 668–70bronchopulmonary dysplasia

108chronic myeloproliferative

disorders 684–6chronic thromboembolic

679–86

classification 662, 662–3, 673congenital heart disease 667–8,

693–7congestive vasculopathy 667,

683–4, 689–91connective tissue disease 392,

697–8, 804COPD 621CREST syndrome 697–8, 822cystic fibrosis 99, 103definition 661drug-induced lung disease

589endothelin-1 392Gaucher disease 410, 412, 414,

698genetics 668histopathologic patterns 672–91,

822hypoxic arteriopathy 686–9idiopathic 661inflammation 670interstitial lung disease 392,

698–700intimal fibrosis in systemic

sclerosis 697Langerhans’ cell histiocytosis

1351leukemia 684–6mechanisms of development

669mixed connective tissue

disease 827non-thrombotic embolic

684–6pathogenesis 668plexogenic arteriopathy 673prognosis 822pulmonary arterioles 664pulmonary capillary

hemangiomatosis 691–3pulmonary tumor thrombotic

microangiopathy 1376pulmonary veno-occlusive

disease 667, 691–3radiological findings 666–8,

667Raynaud’s phenomenon 825rheumatoid arthritis 813risk factors 661sarcoidosis association 392, 492Schistosoma infection 684scleroderma 822, 822serotonin 670sickle cell disease 680Sjögren’s syndrome 825smoking-related disease 392systemic lupus erythematosus

670, 697, 698, 815, 817systemic sclerosis 392, 667,

697–8T cells 675thrombotic arteriopathy

679–86tissue specimen handling 663

vascular disease patterns 673vascular pathology 661vascular remodeling 392,

621, 668vasculitis 700see also idiopathic pulmonary

arterial hypertension(IPAH), plexogenicarteriopathy

pulmonary hypoperfusion696

pulmonary hypoplasia 91–3,93, 94

autopsy 92–3classification 92diagnosis 92–3diaphragmatic hernia 98elastin absence 93fetal breathing impairment 91histology 93idiopathic 92inadequate thoracic volume 91lung:bodyweight ratio 92–3morbidity/mortality 93oligohydramnios 91–2, 93primary 92radial alveolar count 93respiratory distress syndrome

92pulmonary infarction 683–4, 685,

690–1pulmonary interstitium 27–30see also interstitial entries

pulmonary lobule 10pulmonary lymphangio-

leiomyomatosis (PLAM)see lymphangio-leiomyomatosis

pulmonary lymphangiticcarcinomatosis (PLC)1382–4

differential diagnosis 1384pathology 1383–4pathways 1382radiographic findings 1383signs/symptoms 1382–3

pulmonary lymphoepithelioma-like carcinomas (PLELC)956

pulmonary malignant nervesheath tumor 1239

pulmonary mycotoxicosis 439pulmonary neuroendocrine

cells (PNCs) 19–22, 1151,1172

function 20–1hyperplasia 1172, 1173immunohistochemistry 1175neurosecretory vesicle 21–2proliferating 1174, 1175,

1177pulmonary nodular disease

184, 1393see also nodular lymphoid

hyperplasia

Index

1599






pulmonary ossification 55, 392,393, 393

pulmonary papillomatosis 851pulmonary parenchymal diseaseBehçet’s syndrome 750beryllium-induced 540carcinogenesis and scar cancer

theory 910diffuse 366, 366inflammatory bowel disease 834sarcoidosis 478, 480

pulmonary parenchymal tears,Ehlers–Danlos syndrome430–1

pulmonary preinvasive disease889–929

angiogenesis 905–6apoptosis 903basal cell hyperplasia 892–3bronchial lesions 902–9bronchioalveolar lesions 910–11carcinogenesis 909–10, 921–2,

928–9cell immortalization 906genetic factors 929genomic instability 907–8hyperproliferation 903mitosis 903molecular biology 902–9mucus cell hyperplasia 892p53 protein 903–4peripheral lung

adenocarcinogenesis909–19, 921–2

peripheral lung epithelialcarcinogenesis 909–10

progression 908–9, 929sputum atypia 908squamous dysplasia/

carcinoma-in-situ891, 892

squamous metaplasia 891, 892,893–4

transcription factors 906tumor suppressor genes 903–4,

906tyrosine kinase signaling

pathways 905see also atypical adenomatous

hyperplasia (AAH),squamous dysplasia/carcinoma-in-situ(SD/CIS)

pulmonary pseudoisomerism68, 69

pulmonary schwannoma 1239pulmonary sequestration 82–4bronchopulmonary foregut

malformation 85elastic tissue 84extralobar 82–3, 82intralobar 82, 83–4, 84diagnosis 83histology 84management 83–4

isolated systemic arterialsupply 85

mucus retention 84pulmonary teratoma 1285–6cell of origin 1285classification 1285clinical features 1285clinicopathologic correlation

1286differential diagnosis 1286genetics 1285histopathology 1285–6immunohistochemistry 1286macroscopic pathology 1285malignant 1285–6natural history 1286prognosis 1286radiographic features 1285

pulmonary trunk 11pulmonary tumor thrombotic

microangiopathy(PTTM) 1376

pulmonary vascular disease686, 813, 815

pulmonary vasculatureanastomotic complications in

lung transplantation 772–3anatomy 663–6, 664, 665, 666bacterial infection route 150–1

pulmonary vein(s) 11, 30, 30,90, 665

pulmonary vein sarcoma 1243–4cell of origin 1243classification 1243clinical features 1243clinicopathologic correlation

1244cytology 1244differential diagnosis 1244electron microscopy 1244genetics 1243histopathology 1243immunohistochemistry 1244macroscopic pathology 1243molecular findings 1244natural history 1244prognosis 1244radiography 1243

pulmonary veno-occlusivedisease (PVOD) 694

associated conditions 692autoimmune disease

association 691chemotherapy-induced 691clinical features 691connective tissue disease

association 691differential diagnosis 693epidemiology 691hemosiderin deposition 693histopathology 693intimal fibrosis 693medial hyperplasia 693pulmonary hypertension

667, 691–3

radiation therapy-induced 691radiologic features 692–3treatment 691vascular lesions 696

pulmonary venules 11pulmonary–renal syndromes 742pyoderma gangrenosum 832,

832–3, 833pyothorax-associated lymphoma

1357clinical features 1356differential diagnosis 1357EBV association 1356immunohistochemistry 1356molecular pathology 1356pathology 1356, 1356primary effusion lymphoma


prognosis 1356treatment 1356

Q fever 179quercetin 959

radial alveolar count 4, 93radiation pneumonitis 593–4acute 594chronic 594epidemiology 593–4histopathology 594, 594pathogenesis 593with subsequent

chemotherapy 594radiation recall pneumonitis 594radiation therapy 594–5, 595,

691, 1461–2radiation toxicity 593–5radiofrequency ablation 595radon, lung cancer 951RANTES 748, 1412rapamycin/rapamycin analog

toxicity 592rapidly-adapting stretch

receptors (RARs) 12rare earth pneumoconiosis

544–5, 545Raynaud’s phenomenon 698, 825RB1 gene mutations 960reactive nitrogen species (RNS)

1464–5reactive oxygen species (ROS)

157, 1464–5refractory ceramic fibers (RCF)

518, 550, 1439, 1445, 1462regional alveolar damage 382Reid index 614relapsing polychondritis 830,

830–1renal cell carcinoma 1258, 1510metastases 1380, 1397, 1397

Rendu–Osler–Weber diseasesee hereditaryhemorrhagictelangiectasia

respiratory bronchiolitis (RB)385–8

clinical presentation 385diagnostic criteria 388differential diagnosis 386–7histopathology 385–6lung transplantation 790prognosis 388radiological findings 385

respiratory bronchiolitis-associated interstitiallung disease (RB-ILD)367, 385–8

clinical presentation 385diagnostic criteria 388differential diagnosis 386–7histopathology 385–6prognosis 388radiological findings 385

respiratory distress syndrome103–6

air leaks 109, 111diagnostic criteria 104host reaction 104–5incidence 104natural history 104pathology 104pulmonary hemorrhage 111pulmonary hypoplasia 92risk factors 104surfactant therapy 105see also hyaline membrane

diseaserespiratory epithelium 14–16, 153respiratory papillomatosis 850respiratory pathways, collateral

26respiratory syncytial virus 117,

182–3, 191–2bronchiolitis 191, 191diffuse alveolar damage 191multinucleated epithelial

cells 191pathology 191pneumonia 118, 191

respiratory tract cells 14, 15–23,24–8, 30

resveratrol 958retinoblastoma 960pathway 904–5

retinoblastoma (Rb) genemutations 960, 1103, 1115

retinoic acid receptor (RAR) 906retinopathy, paraneoplastic

syndrome 974retroperitoneal fibrosis 1445RGS17 (regulator of G-protein 17)

gene 965rhabdomyomatous hyperplasia

90, 1242rhabdomyosarcomaalveolar 123, 1514–5embryonal 123pulmonary 123–4, 1242

rheumatoid arthritis 806–14

Index

1600






airway disease 811–13amyloidosis 814, 814, 1337,

1338bronchiectasis 813, 813Caplan’s syndrome 808chronic bronchiolitis 813, 813diffuse alveolar damage

pattern 811, 811diffuse alveolar hemorrhage

813empyema 1425eosinophilic pneumonia 814,

814follicular bronchiolitis 812–13interstitial lung disease 808–9lung cancer 814lymphocytic interstitial

pneumonia pattern 811,811

lymphoid hyperplasia 812–13lymphoid proliferation 1317–8lymphoproliferative disorders

814methotrexate-associated

diffuse large B-celllymphoma 814

non-specific interstitialpneumonia pattern809, 810

obliterative bronchiolitis811–12, 812

organizing pneumonia pattern809, 810

pathology 807, 810pleural disease 807, 1425, 1426pleuritis 806pneumothorax 1425pulmonary hypertension 813usual interstitial pneumonia

pattern 809vascular disease 813vasculitis 814

rheumatoid nodules 807–8, 807,808

cavitary 807pathology 807, 808prognosis 808tuberculosis differential

diagnosis 222Wegener’s granulomatosis

differential diagnosis 729rheumatoid pneumoconiosis

(RP) 531, 531, 808, 808rhinosinusitis, lung cancer

risk 957rhinovirus 184Rhodococcus equi pneumonia

173, 178–9Rickettsia conorii pneumonia 179Rickettsia rickettsii pneumonia

173, 174, 179Rickettsiales pneumonia 179right-to-left shunting,

arteriovenousmalformations 1261

right ventricular dysfunction621–2, 697

rituximab toxicity 593river blindness 308rock wool 550Rocky Mountain spotted fever 179Roget cells see pericytesRosai-Dorfman disease 412,

1354, 1354rounded atelectasis 1448–51asbestos count 1451differential diagnosis 1451

rutile 546

S-100 antigen 1165S sign of Golden, squamous

cell carcinoma 1094sagittal slicing 47salivary gland carcinoma,

metastases 1394salivary gland neoplasms

1127–48acinic cell carcinoma 1138–41adenoid cystic carcinoma

1107–8, 1133–6epithelial–myoepithelial

carcinoma 1136–8mucus gland adenoma 1132–3,

1146–8oncocytoma 1148pleomorphic adenoma 1141–4pneumocytic

adenomyoepithelioma1144–5

see also mucoepidermoidcarcinoma

Salmonella pneumonia 174, 179Sanfilippo syndrome 422sarcoid granulomas 464, 481,

482, 483, 483pleural 1424

sarcoidal inclusions 56sarcoidosis 475airway involvement 493angiotensin-converting

enzyme serum levels 489antigen presentation 489antigens 476aspergilloma 482asteroid bodies 485, 485autoimmune disease

association 490berylliosis differential

diagnosis 541biopsy 488bronchiolar 484bronchoalveolar lavage 489calcium oxalate crystals 486cavitary 493classification 479clinical associations 488, 489clinical presentation 478, 492common variable

immunodeficiency 490cutaneous anergy 490

cyst formation 484cytology 487, 487definition 475diagnosis 487differential diagnosis 491diffuse pleural fibrosis


electron microscopy 487end-stage 484environmental triggers 477epidemiology 475epithelioid cells 489ethnic differences 476, 476etiology 476, 477, 477genetics 476geographical distribution 476granulomas 464, 481, 483, 483,

484, 491Hamazaki–Wesenberg bodies

486, 486histopathology 481, 482, 700historical aspects 475HIV infection 490HLA association studies

476, 477hypersensitivity pneumonitis

differential diagnosis 464immune reconstitution

inflammatory syndrome490

immunopathogenesis 489inclusions 485infections 477, 488, 491interferon therapy

complication 490, 492intrathoracic adenopathy 478Kveim–Siltzbach test 489laboratory findings 489lung transplantation recurrent

disease 793–5lymph node involvement 480lymphangitic distribution

482, 488macroscopic pathology 479,

480, 481, 483malignancy association 490natural history 492necrotizing sarcoid

granulomatosis 494, 1424nodular 479, 493, 494, 495pleural 482, 1424pleural effusions 493pregnancy 490prognosis 492pulmonary function tests 478pulmonary hypertension

association 392, 492pulmonary parenchymal

disease 478, 480radiographic findings 478,

479, 480remission rates 492Schaumann bodies 485, 486secondary pneumothorax 1416

smoking 477staging system 480symptoms 478T helper cells 489, 490transbronchial lung biopsy 488transbronchial needle

aspiration biopsy 489treatment 493tuberculosis differential

diagnosis 221variants 493vascular involvement 485Wegener’s granulomatosis

differential diagnosis 729see also necrotizing sarcoid

granulomatosissarcoma 1192metastases 1247, 1400–1see also osteosarcoma, primary

pulmonary osteogenicsarcoma, pulmonaryartery sarcoma, synovialsarcoma

sarcomatoid carcinoma 1186–216classification 1186cytokeratin 7 expression 1020cytology 1194epithelial–mesenchymal

transition 1186–7giant cell sarcoma 1195–7histogenesis 1186–7incidence 1186inflammatory 1193–4, 1193mesothelioma differential

diagnosis 1516–7pleomorphic adenoma 1141–4pleomorphic carcinoma


primary pulmonaryangiosarcoma differentialdiagnosis 1273

primary pulmonary osteogenicsarcoma differentialdiagnosis 1236

sarcomatoid mesotheliomadifferential diagnosis1194–5

spindle cell carcinoma1187–95

thyroid transcription factor 1expression 1018, 1020

transdifferentiation spectrum1187

see also pleuropulmonaryblastoma, pulmonaryblastoma

sarcomatoid carcinosarcoma1516

sarcomatoid mesothelioma1476–7

cadherins 1503calretinin sensitivity 1496–7,

1502–3cytokeratin 5/6 1502

Index

1601






sarcomatoid mesothelioma(cont.)cytokeratin-negative 1496HBME-1 1503histologic variants 1490immunohistochemistry 1500–3primary pulmonary

chondrosarcomadifferential diagnosis1238–9

prognosis 1529sarcomatoid carcinoma


synovial sarcoma differentialdiagnosis 1283

vimentin 1502Wilms’ tumor gene product

1503Sauropus androgynus ingestion

642–3, 643scar cancer theory 910scars/scarring 54Scedosporium infection 238, 241–3clinical features 242–3diagnosis 243epidemiology 241–2incidence 228invasive disease 243mortality 243organisms 241pathology 243prognosis 243treatment 243

Schaumann bodies 55, 56, 541sarcoidosis 485, 486

Scheie syndrome 422Schistosoma (schistosomiasis)

322–6acute 324chronic 325clinical features 324diagnosis 325–6egg 323, 324epidemiology 322–4life cycle 323necrotizing granulomas 325organism 323–4, 324pathology 325plexogenic arteriopathy 684pulmonary hypertension 684pulmonary vascular disease 686radiographic appearance 325treatment 326

scleroderma see systemicsclerosis

sclerosing hemangioma 864–72alveolar adenoma differential

diagnosis 864cell of origin 864–5cell types 868classification 864–5, 867clinical features 865clinicopathological correlation

871–2

cuboidal cells 869, 870cystic pattern 866cytology 869–70, 869, 870differential diagnosis 872electron microscopy 870–1etiology 864–5genetics 865histopathology 866–9, 868immunohistochemistry 870, 871lung adenocarcinoma

differential diagnosis 1065macroscopic pathology 865–6,

865, 866metastases 869molecular findings 871multiple 866, 867natural history 872neuroendocrine markers 870neuroendocrine proliferations

866–7papillary adenoma differential

diagnosis 861papillary pattern 866, 869pathogenesis 864–5prognosis 872radiographic findings 865sex steroid hormone receptor

expression 870solid pattern 866, 869stromal metaplasia 868thyroid transcription factor 1

expression 1019scrub typhus 179sea-blue histiocytes 416–17secretoglobin 1a1 (Scgb1a1) 7sectioning techniques 46–7segmentectomy 45–7semaphorins 905senile lung 623senile pulmonary amyloid (SSA)

32, 56sensory receptors 12sepsis, hematogenous spreadCandida 265pneumonia-causing organisms

150, 151–2septic emboli 150, 151–2seromucinous glands 15, 16, 23serotonin, pulmonary

hypertension 670serous borderline tumor of

paratestis 1473serous cells 18–19serous papillary carcinoma of the

ovary 1512SERPINA1 gene mutations

606, 619Serratia marcescens pneumonia

160, 175serum mesothelin-family

proteins (SMRP) 1454severe acute respiratory syndrome

(SARS) 182, 190sex cord stromal tumors,

metastases 1398

Sézary syndrome 1346Shp2 tyrosine protein

phosphatase 6shrinking lung syndrome 819shrinking pleuritis with rounded

atelectasis see folded lungsialomucins, cell-associated

1409sickle cell disease 681acute chest syndrome 680, 681chronic lung disease 680hypoxia 680pulmonary hypertension 680

siderosilicosis 544silicabiologic activity 528exposure 528mineralogy 528occupations causing exposure

528particle size 528tissue injury mechanisms 528

silicates 528, 536, 538, 1445silicatoses, non-asbestos 536–40silicon carbide pneumoconiosis

544, 545silicoproteinosis, acute (ASP)

530–1, 531silicosis 528alveolar lipoproteinosis 530–1bronchogenic carcinoma

association 531–2clinical features 528complicated 529conglomerate 529diagnosis 529emphysema 530endobronchial 633–4extrathoracic lesions 530fibrosis 529immune dysfunction 531–2latency period for

development 528Mycobacterium tuberculosis

infection 529nodular 529, 529, 530, 530pathogenesis 528pathologic features 529–31radiologic features 528–9, 529rounded atelectasis 1451

silicotic lung disease 528–32silo-unloader’s syndrome 439simian virus 40, 1463

mesothelioma 1462–3single gene polymorphisms,

lung cancer risk 960–3sirolimus toxicity 592, 791, 1255Sjögren syndrome 823–5amyloidosis 1338, 1339autoantibodies 823, 825bronchi/bronchioles 824–5chronic bronchiolitis 825diagnosis 823interstitial lung disease

823–4

lymphocytic interstitialpneumonia 823–4,1319, 1320

lymphoid hyperplasia 824, 825lymphoid proliferation 1317–8marginal zone B-cell

lymphoma 825nodular lymphoid hyperplasia

825non-Hodgkin’s lymphoma 825nonspecific interstitial

pneumonia pattern823, 824

organizing pneumonia pattern824

pathology 823primary 823pulmonary hypertension 825secondary 823usual interstitial pneumonia

pattern 824slab technique 46–7SLC34A2 gene mutation 394sleeping sickness

see Trypanosoma brucei(African trypanosomiasis)

sleeve lobectomy 46, 46sling left pulmonary artery 69slowly-adapting stretch receptors

(SARs) 12small airway diseases 636–48, 637classification 637–8clinical features 636–7definition 636inflammatory bowel disease

833–4see also bronchiolitis, diffuse

panbronchiolitis (DPB),follicular bronchiolitis,peribronchiolarmetaplasia

small cell carcinoma 1153extra-pulmonary 1020, 1022

small cell lung carcinoma(SCLC) 889, 909, 949,1162

Azzopardi phenomenon1162–3, 1163

basaloid carcinoma differentialdiagnosis 1118


carcinoid tumor differentialdiagnosis 1169

classification 1151–2clinical features 1153combined 1093, 1164crush phenomenon 1162–3,

1167cytology 978, 1162, 1163–4,

1163, 1165dense core vesicles 1151differential diagnosis 1167–8dissemination 1153

Index

1602






electron microscopy 1166epidemiology 1153genetics 1166–7histopathology 1162–4incidence 949intra-pulmonary thymoma


large cell neuroendocrinecarcinoma differentialdiagnosis 1168–9, 1169

lymphoid infiltrate differentialdiagnosis 1028

macroscopic pathology 1156,1156–7, 1162

marginal zone lymphomadifferential diagnosis1328–9

metastases 1153natural history 1169neuroendocrine markers 1027non-endocrine component

1164paraneoplastic effects 1153prognosis 1169pulmonary blastoma


radiographic findings 1156,1156

small cell variant of squamouscell carcinoma differentialdiagnosis 1106–7, 1029

squamous cell carcinomacombination 1093differential diagnosis 1031

staging 1169subtypes 1151–2thyroid transcription factor 1

expression 1016, 1018,1022

TNM classification 1012small specimen histological

processing 42–3description 42fixatives 42handling 42identification 42media 42molecular testing 43, 50–1pathological finding

correlation with clinicalfindings 42–3

sample collection 42tissue amounts 43

small vessel remodeling inthrombotic arteriopathy683

small vessel vasculitis 622smokingacute eosinophilic pneumonia

579asbestos exposure and lung

cancer 525, 526, 953asbestosis 520, 525, 1439–40

atypical adenomatoushyperplasia association921, 926–7

bidi composition 950bronchial carcinogenesis 891bronchiolitis 644bronchus-associated lymphoid

tissue 15carcinogenic effects 900carcinogens 950, 951carcinosarcoma 1198cessation 950chromosome 3p changes 907cigarette composition 950coal dust exposure 536COPD 609–11emphysema 620, 621epidemiology 947giant cell sarcoma 1196goblet cell metaplasia 565hypersensitivity pneumonitis 449irritant effects 900Langerhans’ cell histiocytosis

1349, 1351lesions in lung transplantation

790, 791lung adenocarcinoma 1066lung cancer association 890–1,

946, 947–8, 950–1macrophage production

27, 385p16 gene 907passive 950pleural plaques 1439–40prevalence 929pulmonary blastoma 1207pulmonary hypertension 392rates 946sarcoidosis 477squamous cell carcinoma 1094squamous cell papilloma

association 850squamous dysplasia/

carcinoma-in-situ 891, 900squamous metaplasia 893–4see also respiratory

bronchiolitis (RB),respiratory bronchiolitis-associated interstitial lungdisease (RB-ILD)

smoking-related interstitial lungdisease 385

smooth muscle 23hyperplasia 54–5, 55

smudge cells, adenovirus 194, 194soft tissue sarcoma, metastases

1400solid fuel use, lung carcinoma 946solitary fibrous tumor of the

pleura 1429–36benign area transition to

malignant area 1434calcifying fibrous tumor

of the pleura differentialdiagnosis 1429–30

cell of origin 1430–1classification 1436clinical features 1431clinicopathological correlation

1435–6cytology 1433differential diagnosis 1435electron microscopy 1433genetics 1431hemangiopericytoma-like

pattern 1433histopathology 1432hypertrophic pulmonary

osteoarthropathy 1431hypoglycemia 1431immunohistochemistry 1433,

1435incidence 1430keloid-type area 1433macroscopic pathology 1431malignant potential 1434mesothelioma differential

diagnosis 1518molecular findings 1433–5natural history 1436paraneoplastic syndromes 1431patternless pattern 1433prognosis 1436pseudorosette area 1434radiology 1431treatment 1436

Sparganum mansoni(sparganosis) 334–5

specimen handling 41–62ancillary studies 49–51autopsy 45–7bronchoplastic resection 45–7cytology specimen processing

41–2electron microscopy 50histochemical stains 49immunohistochemistry 49–50incidental lesions 53, 53–9large surgical pathology

specimen processing43–9

lung carcinoma large specimenevaluation 47–9

molecular techniques 43, 50–1pneumonectomy 45–7segmentectomy 45–7simple lobectomy 45–7small specimen histological

processing 42–3see also biopsy

sphingolipidoses 409sphingomyelin 414, 416sphingosine-1-phosphate (S1P) 18spindle cell carcinoma 1187–95carcinosarcoma differential

diagnosis 1204clinical findings 1187cytology 1188definition 1187differential diagnosis 1192–5

electron microscopy 1190histopathology 1188immunohistochemistry

1188–90macroscopic pathology 1187molecular findings 1190–2natural history 1195prognosis 1195radiographic findings 1187

Splendore-Hoeppli 233, 273, 310,324, 325

spindle cell sarcoma 864, 1517–8sponge artefact, biopsy 52, 53Sporothrix schenckii

(sporotrichosis) 272–4, 273clinical features 272culture 274diagnosis 273–4differential diagnosis 274epidemiology 272organism 272pathology 272–3prognosis 274serological tests 274South American blastomycosis

differential diagnosis 264systemic 272treatment 274

sputumatypia 908cytology processing 41–2microbiological workup 154

squamous cell(s), cytologicalatypia 891–2

squamous cell carcinoma 949,1093–108

aberrant methylation of CpGislands 1103–4

acantholytic 1193age factors 1094alveolar filling type 1105,

1097–8amyloid-like stroma 1101atypical carcinoid tumor


basaloid variant 1107–8, 1108,1118

carcinosarcoma 1198malignant spindle cell stromalcomponent 1188, 1192

small cell lung carcinomadifferential diagnosis1167–8

cavitation 1094, 1095, 1105cell of origin 1093central tumors 1095–6, 1097,

1098classification 1093clear cell 1106, 1106, 1107clinical features 1094clinicopathologic correlation

1104CT scanning 1095cystic change 1098

Index

1603






squamous cell carcinoma (cont.)cytology 1100–2, 1102, 1103desmosomes 1103diagnosis 1104differential diagnosis 1104–5electron microscopy 1103epidermal lysosomes 1103epigenetic modifications

1103–4gender 1094gene deletions 1093–4genetics 1093–4granular cell tumor differential

diagnosis 1242high grade 1099histone modification 1104histopathology 1106, 1096–9,

1097, 1098, 1099human papilloma virus

association 906, 1098–9immunohistochemistry 1101,

1103, 1099incidence 889, 949, 1093intrathoracic spread 1094keratin gene expression 1104keratinizing 1101lung adenocarcinoma


macroscopic pathology1095–6, 1096

metastases 1094microRNAs 1104, 1105molecular findings 1103–4morpheaform invasion 1101morphologic variants 1099Napsin A positivity 1023natural history 1105non-keratinizing 1101–2, 1102occult of bronchus 1094Odland bodies 1103papillary 1105–6, 1105, 1106paraneoplastic syndromes 1094pathogenesis 1093peripheral lung 1105, 1094–5,

1096, 1096, 1097–8,1099, 1100

PET-CT scanning 1095primary of pleura 1509prognosis 1105pseudoangiomatous 1273pseudoglandular 1101radiographic findings 896,

1094–5, 1095S sign of Golden 1094small cell lung carcinoma

1031, 1093small cell variant 1106–7, 1107,

1029smoking 1094squamous cell papilloma

differential diagnosis 855squamous dysplasia/

carcinoma-in-situassociation 908

staging 1094surgery 1105thyroid transcription factor 1

expression 1016, 1018tonofilaments 1103treatment contraindications

1104tumor growth 1103variants 1105–8well-differentiated 1098–9

squamous cell metaplasia 899squamous cell papillomas 850–5alveolar parenchymal

involvement 853–4classification 850clinical features 850clinicopathologic correlation

855cytology 854, 854–5differential diagnosis 855electron microscopy 855etiology 850histopathology 851–4human papilloma virus 850,

852–3, 855immunohistochemistry 855inverted 853, 853–6macroscopic pathology 851malignant transformation 855mixed glandular papilloma

856, 857multiple 851natural history 855papillary squamous cell


pathogenesis 850pathology 853, 854prognosis 855radiographic findings 850–1smoking association 850solitary 851, 851, 852, 852–3squamous cell carcinoma


squamous dysplasia/carcinoma-in-situ (SD/CIS) 898, 889,894–902

angiogenic 899autofluorescence bronchoscopy

896–7, 908–9basement membrane thickness

898bcl2 protein 904bronchial clearance

compromise 895bronchial epithelium 900CD44v6 906chromosomal aneusomy 907classification 895, 900concurrent squamous cell

carcinoma association 908cytology 899detection 891–2, 896–7diagnosis 898–9, 900, 903

differential diagnosis 900DNA aneuploidy 907environmental irradiation 892etiology 890–2exfoliative cytology 898–9grades of disease 897, 898, 899histologic assessment 900histologic features 895hyperproliferation 903lesion size 895–6macroscopic pathology 895–7microscopic features 897–8mild dysplasia 897, 897, 899,

900–1moderate dysplasia 897, 897,

899neovascularization 898p53 status concordance 904pre-invasive bronchial

lesions 892progression risk 908, 909, 909retinoic acid receptor function

loss 906severe dysplasia 897, 898, 899,

902, 902small cell lung carcinoma

association 909smoking 891, 900squamous metaplasia 891, 892,

894telomerase reverse

transcriptase 906WHO diagnostic criteria 900

squamous epitheliumatypical 901reactive 902

squamous metaplasia 893,893–4, 894

diffuse alveolar damage 348factors associated with 894non-dysplastic 894smoking 893–4squamous dysplasia/

carcinoma-in-situ 891,892, 894

squamous papillary lesions,atypical 901

stannosis 546Stanton hypothesis,

mesothelioma 1467Staphylococcus 152, 176non-coagulase producing 176

Staphylococcus aureus 172abscess 163botryomycosis 164, 176cystic fibrosis 99pneumonia 148, 176

Starling forces, pleural fluid 1409starvation, emphysema 623–4status asthmaticus 565stem cells, lung development 7STK11/LKB1 gene mutation,

lung adenocarcinoma1063

Streptococcab pneumonia 176–7

Streptococcus, hemolytic 164, 165Streptococcus agalactiae

pneumonia 176Streptococcus pneumoniae 147,

153, 164, 177Streptococcus pyogenes 177Streptococcus viridans group

pneumonia 177Strongyloides stercoralis

(strongyloidiasis)316–18, 318

Churg–Strauss syndromedifferential diagnosis 736

clinical features 317diagnosis 317–18epidemiology 316organism 317pathology 317treatment 318

subacute sensory neuropathy,lung carcinoma 973–4

submucosa 15elastosis 54

submucosal glands 23cystic fibrosis 99

subpleural fibrosis 370respiratory bronchiolitis/

RB-ILD 386, 387subpleural lobules 10succinated dehydrogenase

missense mutation,primary pulmonaryglomus tumors 1249

sugar tumor see clear cell tumorof the lung (CCTL)

superficial spreading 1095–6superior vena cava syndrome

970, 1047supernumerary arterial branches

663, 664–5, 666surfactantdeficiency in respiratory

distress syndrome103–4

metabolic dysfunction 106phospholipids 25–6secretion by type II

pneumocytes 25–6synthesis 6

surfactant apoprotein A 1025surfactant protein(s) 4, 26, 1025,

1026surfactant protein A (SP-A) 26,

103–4atypical adenomatous

hyperplasia 925idiopathic pulmonary fibrosis

958immunohistochemistry 1025,

1026lung cancer 958pleomorphic carcinoma 1189variations associated with

respiratory distresssusceptibility 106

Index

1604






surfactant protein B (SP-B) 26,103–4, 1025

absence 106variations associated with


surfactant protein C (SP-C) 6, 7,26, 103–4

mutations 368variations associated with


surfactant protein D (SP-D)26, 103–4, 428

surfactant therapy, pulmonaryhemorrhage 111

surgical lung biopsy, interstitiallung disease 366

survivin, atypical adenomatoushyperplasia 923

Sweet’s syndrome 833Swyer–James–MacLeod

syndrome 626, 639, 640synaptophysin 1026, 1027, 1164,

1170syndrome of inappropriate

antidiuretic hormonesecretion (SIADH)971, 1153

synovial sarcoma 124, 1281–3biphasic 125, 1516cell of origin 1281classification 1281clinical features 1281clinicopathologic correlation

1283cytology 1283differential diagnosis 1283electron microscopy 1283histopathology 1282–3immunohistochemistry 1283macroscopic pathology 1281–2mast cells 1283mesothelioma differential

diagnosis 1516metastatic 1283molecular findings 1283monophasic 125monophasic spindle cell

tumors 1282–3, 1435natural history 1283pleomorphic carcinoma

differential diagnosis 1193pleural 1517poorly differentiated 1516prognosis 1283radiographic findings 1281

synthetic vitreous fibers 1439,1445, 1462

syphilis 179, 221systemic arterial supply, isolated 85systemic inflammatory response

syndrome (SIRS) 262systemic lupus erythematosus

(SLE) 814–20

acute lupus pneumonitis 815antiphospholipid syndrome

815, 819–20capillaritis 742diffuse alveolar damage 816diffuse alveolar hemorrhage

815–17, 815, 816drug reactions 819interstitial lung disease 817–19lymphocytic interstitial

pneumonia 816nonspecific interstitial

pneumonia pattern 818organizing pneumonia pattern

819pathology 815pleural disease 1425–6pleurisy 1425pleuritis 815pulmonary hemorrhage

syndrome 740pulmonary hypertension 670,

697, 698, 815, 817shrinking lung syndrome 819thromboembolic disease 818vascular disease 815vasculitis 816

systemic sclerosis 820–3autoantibodies 820–1clinical manifestations 820interstitial lung disease 821–2lung cancer 822–3, 958nonspecific interstitial

pneumonia pattern821–2, 821

patchy congestion 698pathology 821pulmonary hypertension 392,

667, 697–8, 822, 822usual interstitial pneumonia

pattern 821–2vascular disease 699

T-cell lymphomas 1343, 1346T-cell prolymphocytic leukemia

1347T-cell rich large B-cell lymphoma

1334T cellsasthma 566Churg–Strauss syndrome 735immune response 154mycobacterial infections 213pulmonary hypertension 675

T helper 2 (Th2) cells 373T helper cells 489, 490Taenia solium (cysticercosis)

333–4clinical features 333diagnosis 334epidemiology 333organism 333pathology 334

tail sign, lung adenocarcinoma1047

Takayasu arteritis 747–9clinical manifestations 747–8diagnosis 747–8differential diagnosis 748epidemiology 747genetics 747giant cell arteritis differential

diagnosis 747, 748histopathology 748laboratory findings 748macroscopic pathology 748natural history 748–9pathogenesis 748prognosis 748–9pulmonary artery involvement

748radiographic findings 748tuberculosis association 748

talc embolism 537talc granulomatosis 537, 600talcosis 536–8, 1417asbestos contamination 536–7,

538pathology 537progressive massive fibrosis

537radiological appearance 537

telomerase reverse transcriptase,human (hTERT) 906, 925

tension pneumothorax 110teratomamediastinal 1286see also pulmonary teratoma

terminal respiratory unit (TRU)910

testicular non-seminomatousgerm cell tumor,metastases 1381, 1399

testicular seminoma, metastases1399

tetralogy of Fallot, pulmonaryhypertension 693–5, 696

TFE3 expression, clear cell tumor1258

TGF-p type II receptor (TpRII) 6thoracic aorta, large vessel arising

from 83thoracic duct trauma 1428thoracic volume, inadequate 91Thorotrast, peritoneal

mesothelioma 1461thrombo-embolism/thrombo-

embolic disease 682antiphospholipid syndrome 820chronic recurrent 768genetic factors 679–80lung infarct 685post-thrombotic remnants 683pulmonary artery occlusion

680pulmonary hypertension

666–7systemic lupus erythematosus

818thrombosis 112, 680

thrombotic arteriopathy 679–86,687

associated conditions 679congestive vasculopathy 683–4differential diagnosis 684–6epidemiology 679–80fibrotic remnants 682–3histopathology 680–3pulmonary infarcts 683–4risk factors 679–80small vessel remodeling 683thrombus organization 680–2vascular lesions 681

thrombusepithelioid granulomas 682organization 680–2

thymic tumors 1510–2thymomaintra-pulmonary 1283–5mediastinal 1285pleural 1511, 1512

thyroid carcinomametastases 1395thyroid transcription factor 1

expression 1019see also papillary thyroid

carcinomathyroid transcription factor 1

(TTF1) 910, 1015clones 1024cytoplasmic staining 1023embryology role 1015expression 1015, 1016carcinoid tumor 1018, 1019,1165

extra-pulmonaryneuroendocrine tumors1022

extra-pulmonary small cellcarcinoma 1020, 1022

large cell carcinoma 1016,1019


lung tumors 1016non-pulmonary tumors1019, 1021

sarcomatoid carcinoma1018, 1020

small cell lung carcinoma1016, 1018

small cell lung carcinomaand Merkel cellcarcinoma of skincomparison 1022

squamous cell carcinoma1016, 1018


lung cancer prognosis 1020mesothelioma and lung

adenocarcinomadistinction 1495–6

monoclonal antibodycomparison 1021

Index

1605






thyroid transcription factor 1(TTF1) (cont.)mutations 1016neuroendocrine tumors 1165NKX2–1 amplification 1064nuclear staining 1023pleomorphic carcinoma 1189squamous cell carcinoma


structure 1015tin exposure 546titanium, pleural plaques 1439titanium pneumoconiosis

545–6, 548TNM classification 48fixed tissue/fresh tissue 48pleural invasion 48

Toll-like receptor(s) (TLRs)153, 229–30

Toll-like receptor 7 (TLR-7) 28Toll-like receptor 9 (TLR-9) 28tongue carcinoma, metastases

1388tonofilaments, squamous cell

carcinoma 1103Toxocara (toxocariasis) 317, 319,

319–20, 736clinical features 319diagnosis 320epidemiology 319organism 319pathology 319–20, 320

Toxoplasma gondii(toxoplasmosis) 301–4

clinical findings 301–2congenital 301–2diagnosis 302–3differential diagnosis 303epidemiology 301hosts 301human infection 301immunohistochemistry 303interstitial pneumonitis 302life cycle 301necrotizing pneumonia 302organism 301pathology 302, 303radiographic appearance

302, 302reactivation of latent infection

302treatment 303–4

trabeculae 10tracheaanatomy 7–8congenital malformations

66–71development 1, 4, 6fibrous webs 71growth 6length abnormalities 66mucus retention cysts 71organization 7–8

tracheal agenesis 66–7, 67

classification 67diagnosis 67embryology 67malformation patterns 67presentation 67

tracheal stenosis 67–9, 71age 69associated abnormalities 69classification 67diffuse 68, 68extrinsic 69funnel-shaped 68–9intrinsic 68–9mortality 69segmental 69symptom severity 69vascular anomalies 69

tracheoesophageal fistula 70–1, 71associated malformations 70etiology 70outcomes 71subtypes 70–1

tracheobronchial cartilage 53–4tracheobronchial papillomatosis

854tracheobronchial sicca 824–5tracheobronchomegaly 71, 430tracheobronchitis, viral infections

182, 184, 185–6tracheobronchopathia

osteochondroplastica635–6, 636

tracheobronchopathiaosteoplastica 71

tracheomalacia 69–70, 70traffic-related pollution 952transbronchial lung biopsy (TBBx)

59–60, 769–70, 774efficacy 60, 60–1high utility 60–1probably/possibly diagnostic 61reliability 60, 60–1unreliable uses 61

transcription factors 5, 906transforming growth factor b

(TGF-b) 428, 429, 1467transforming growth factor b

(TGF-b) receptor 2mutations 428

transforming growth factor b1(TGF-b1) 6

transfusion-related acute lunginjury (TRALI) 595–6

transplant-associatedvasculopathy (TAV)792–3

trastuzumab toxicity 593TREM-1 153–4, 1412trematodes 322–8food-borne 328

tremolite, environmental pollution1437–8, 1459–60

Treponema pallidum 174, 179triazole, Aspergillus infection

238–9

Trichomonas 307Trichophyton rubrum 228Trichosporon, Japanese summer-

type hypersensitivitypneumonitis 459

triggering receptor expressedon myeloid cells-1(TREM-1) 153–4, 1412

trisomy 21, development-relatedairspace enlargement 623

Tropheryma whipplei (Whippledisease) 174, 179, 413

tropical pulmonary eosinophiliasyndrome 308–11, 311

clinical features 309diagnosis 310–11epidemiology 308organism 308–9pathogenesis 311pathology 309–10

Trypanosoma brucei (Africantrypanosomiasis) 295,296, 297

Trypanosoma cruzi (Chagasdisease) 290–1, 295–7,296

clinical features 295diagnosis 295differential diagnosis 295–7epidemiology 295organism 295pathology 295transmission 295vector 295

TSC gene mutations 859, 926,1249–50, 1254

tuberculin skin test 209tuberculoma 216, 217tuberculosis see Mycobacterium

tuberculosis(tuberculosis)

tuberculous bronchopneumonia217, 217

tuberculous empyema 1424tuberous sclerosis complexangiomyolipoma 1249, 1250clinical features 857–8genetics 857, 926lymphangioleiomyomatosis

1249–50, 1252micronodular pneumocyte

hyperplasia 917multifocal micronodular

pneumocytehyperplasia 857

tuft cells see brush cellstularemia 178tumor emboli 1389–90differential diagnosis 1390histopathology 1389–90location 1389macroscopic pathology 1389non-thrombotic 688pulmonary embolism 1389radiographic findings 1389

tumor suppressor genesmesothelioma 1466, 1467pulmonary preinvasive disease

903–4, 906tumorlets 21, 1172–3, 1176–7,

1177, 1229tumour necrosis factor a (TNFa)

213tungsten carbide

pneumoconiosis 541–3tyrosine kinase inhibitorsresistance 1062toxicity 592, 593

tyrosine kinase signalingpathway 905, 923–5

ulcerative colitis 833–5ultrastructure of lungs 13–17upper respiratory system 7upstream regulatory factors

(USF) 906uranium miners, lung cancer

891, 953urinothorax, pleural effusion

1411urothelial carcinoma, metastases

1383, 1393usual interstitial pneumonia (UIP)

114, 367, 368–74, 524abnormal tissue repair 373–4apical cap differential

diagnosis 1423apoptosis abnormalities 374asbestosis differential

diagnosis 519, 521–2associated conditions 369bronchoalveolar lavage 369chronic eosinophilic

pneumonia 572, 573clinical presentation 368–9connective tissue disease

371, 804cytokine polymorphisms 374dermatomyositis 826desquamative interstitial


differential diagnosis 370–2discordance in diagnosis 371drug-induced lung disease

587–8etiology 368fibroblastic foci 375fibrotic interstitial pneumonia

384Hermansky–Pudlak syndrome

differential diagnosis 421histologic features 372hypersensitivity pneumonitis

455, 457, 460–3differential diagnosis 445

immune system 373lymphangioleiomyomatosis


Index

1606






lymphoid proliferation 1318natural history 374neutrophils 373pathogenesis 372–4polymyositis 826prognosis 374radiological findings 369rheumatoid arthritis 809scleroderma 821–2Sjögren syndrome 824superimposed histological

patterns of lung disease371

see also idiopathic pulmonaryfibrosis (IPF)

uterine carcinoma, metastases1384, 1398

uterine leiomyoma 1247, 1398uterine leiomyosarcoma,

metastases 1398uterine tumors, metastatic

878–9

vaccinationmeasles 193viral infections 183

VACTERL (Vertebral anomaly,Atrial atresia, Cardiac,Tracheo-Esophagealfistula with atresia,Renal anomalies andLimb abnormality)spectrum 67

bronchomalacia 72–3horseshoe lung 75lung agenesis 75tracheoesophageal fistula 70

vacuum chamber formalinfixation, wedgebiopsy 45

Varicella pneumonia 183, 199,200

varicella zoster virus (VZV)197–8

vascular cell adhesion molecule 1(VCAM-1) 680

vascular endothelial growthfactor (VEGF) 5, 109,620, 864, 905–6

vascular endothelial growthfactor 1 (VEGF-1) 677

ANCA-associated diseases711–40

autoimmune disease 700Behçet’s syndrome 749–52capillaritis 740–5categorization 712Churg–Strauss syndrome

734, 736classification 711definition 711diagnosis 700drug-induced lung disease

589giant cell arteritis 745–7

necrotizing sarcoidgranulomatosis 495

polyarteritis nodosa 700, 743–5pulmonary hemorrhage

syndromes 740–5pulmonary hypertension 700rheumatoid arthritis 814systemic lupus erythematosus

816Wegener’s granulomatosis 720

vasculogenesis 4, 5veno-occlusive disease 589venous thromboembolism

679–80, 975ventilationcomplications 111see also mechanical ventilation

ventilation/perfusion mismatch,meconium aspiratesyndrome 112–13

vermiculite 539–40video-assisted thoracoscopic

surgery (VATS) 43,49, 977

biopsy 59–60, 770vimentin, sarcomatoid

mesothelioma 1502viral infections 116–18bacterial superinfections 185,

189–90bronchiolitis 185–6cellular changes 187diffuse alveolar damage 184,

186–7epidemiology 182–4fungal superinfections 185hematogenous dissemination

184host factors 183human 182injury patterns 186lung carcinoma 953–6lung injury pattern 185lung transplantation 770, 783–5pathology 182–202pathophysiology 184patterns of disease 182pneumonia 147predisposing factors 182pulmonary defenses 184–5radiographic appearance 184seasonality 184tracheobronchitis 182, 184,

185–6transmission 183–4vaccination 183see also named viruses and

diseasesvisceral larva migrans 308, 316,

318–20vitamin A deficiency 894, 958–9vitamin C 959–60vitamin D 959–60vitamin E 959–60volatile inhalant abuse 600

volume doubling time (VDT),lung carcinoma 968

von Hippel–Lindau disease 861von Recklinghausen’s disease

1239voriconazole 239, 243, 244

Waldenström’smacroglobulinemia 1331

Warthin–Finkeldey cell 193wedge biopsyfixation 44–5formalin fixation 45frozen section 44indications 43multiple lobe sampling 44nodular density 44open 43–4processing 43–4, 45

Wegener’s granulomatosis713–29

A1AT deficiency association714

active disease 728acute eosinophilic pneumonia

differential diagnosis 580acute interstitial pneumonia

differential diagnosis 382age effects 714airspace disease 717airway lesions 727airway necrotizing lesions 715airway wall thickening 717alveolar hemorrhage 727ANCA 729, 834–5antigens 712, 728subtypes 714testing 713, 728

autoantibodies 711, 728bronchiolitis 718capillaritis 742Churg–Strauss syndrome


clinical manifestations 714,714–15

collagen necrosis 720cytology 728differential diagnosis 728–9epidemiology 713–14filarial disease differential

diagnosis 310–11fulminant 727genetics 714giant cell arteritis differential

diagnosis 747Goodpasture’s syndrome

overlap 739–40granulomatous bronchiolitis

645–6granulomatous inflammation

724–5, 726–7granulomatous necrosis 724,

725hemorrhage 718, 727

histopathology 718–28historical aspects 713imaging techniques 715incidence 713–14infections 715, 728inflammation 728inflammatory bowel disease


laboratory findings 728linear densities 717lymphadenopathy 717lymphomatoid granulomatosis


macroscopic pathology718, 719

malignancy risk 715mediastinal masses 717microabscesses 723–4, 723,

724microscopic polyangiitis


natural history 729necrotizing sarcoid

granulomatosisdifferential diagnosis 498

neutrophils 728nodules 715–17, 718, 718organ involvement 720palisading granuloma

725, 726palisading histiocytes 725–6pathogenesis 728, 729pleural disease 1424, 1424–5,

1425polyarteritis nodosa


prognosis 729pulmonary endometriosis

differential diagnosis 879pyoderma gangrenosum


radiographic findings 715–18,716, 717

splenic 719superficial protracted phase

715tissue necrosis 720, 723, 724treatment 727, 729tuberculosis differential

diagnosis 221tumorous masses 727upper respiratory tract

involvement 714, 720vasculitis 720, 722, 723

weight loss, COPD 622–3welder pneumoconiosis 547–9aluminum fumes 548–9lung cancer association 549pathology 548, 548, 549radiographic appearance 548

Index

1607






well-differentiated fetaladenocarcinoma(WDFA) 878–9

Werner’s syndrome 960Westermark sign 666–7, 667Whipple disease 174, 179, 413Wilms’ tumor 1376–7, 1389

Wilms’ tumor gene product(WT1) 1497–8, 1503

Wilson–Mikity syndrome 109wood smoke exposure 553Wucheria bancrofti 308–9dirofilariasis differential

diagnosis 313

eosinophilic pneumonia 310life cycle 310microfilaria 310

xenobiotic metabolizing enzymes(XMEs) 891, 907, 926,929

xenobiotics 950xerotrachea 824–5

Yersinia pestis 175, 179

zebra bodies 418, 422, 426zeolites 538–9, 1437

Index

1608






Documents

Spencer’s Pathology of the Lungassets.cambridge.org/97805215/09954/index/... · adenomatous rete testis hyperplasia 1473 adenosquamous carcinoma 1121–3, 1123 cell of origin 1121