History of the Penrose Cancer Conference · Jochelson, CA Forscher 1995 Prostate and Testicular Cancer Jl Epstein, RD McLeary, JA Smith, Jr., TM Ulbright, AT Stavros ... 25 East Jackson

(0 f I History of the Penrose Cancer Conference

In the early 1940's, pathologist Lauren Ackerman and oncologist/radiotherapist Juan del Regato, both from Ellis Fischel Cancer Hospital in Columbia, Missouri, published their classic book, CANCER. Diagnosis and Treatment. Their book became the foundation for informal conferences attended by such medical notables as Arthur Purdy Stout, the acknowledged founder of surgical pathology as a specialty. Dr. del Regato often discussed clinical, radiologic and radio-therapeutic data with visiting professionals, laying the groundwork for future cancer conferences.

In January of 1949, at the request of Julie Penrose, Dr. del Regato became the Director and Radiotherapist at Penrose Cancer Hospital, a part of The Glockner-Penrose Hospital. Dr. del Regato decided to develop a more formal conference, primarily for pathologists from the Rocky Mountain area. Although not a pathologist himself, he felt that the Conference would have a more universal appeal with this focus. Under the cosponsorship of the Colorado Society of Clinical Pathology, the Penrose Cancer Seminar debuted on September 10, 1949 in the Little Theater of the Broadmoor Hotel.

Prior to the first formal conference, the only one which focused on a variety of cases rather than on one organ or one major disease, Dr. del Regato sent slide sets of 16 cases to 120 pathologists for their opinions. Conference faculty, Arthur Purdy Stout, M.D., and Lauren Ackerman, M.D., jointly discussed breast, skin, salivary gland, etc. cases. Drs. Stout and Ackerman disagreed on the diagnosis of only three out of the 16 cases submitted. Diagnoses from the other pathologists were more varied. One of the purposes of the Conference, which drew over 500 participants, from both coasts, was to show how frequently good pathologists justly differ in the diagnoses of difficult cases on the basis of morphology alone. Proceedings of the Conference were published in Cancer Seminars, Vol. I, No. I, September 1950.

The second seminar, held on September 9, 1950, concentrated on bone tumors. Dr. Phillip Hodes, Professor of Radiology at the University of Pennsylvania was added to the faculty and continued the following year in that capacity. Dr. Ackerman served as pathologist for this seminar as well as for the third and fourth events. From that point on, pathologists and radiologists equally shared in conference presentations.

At the sixth conference in 1954, a clinician was added. Usually a surgeon, this new faculty component added yet another dimension to the conference. Since 1954, pathologists, radiologists and clinicians serving as guest speakers have been national leaders in their specialties.

The conferences continued annually at the Broadmoor Hotel. As attendance grew, their location moved from the LitUe Theater to the Ballroom and finally to the International Center. Dr. del Regato led 24 seminars until his departure from Penrose Hospital in 1972. The Pathology Department continued to coordinate the Conference for an additional five years, until they ended the conferences in 1977.

The Penrose Cancer Seminars were unique. They united the country's premiere pathologists and radiologists in a forum to discuss perplexing problems in diagnosis.

Opinions of the guest speakers, other world experts. and numerous attendees were published annually. The contributions and failures of both tissue morphology and imaging techniques, as well as the utilization of all modalities to arrive at a correct diagnosis, were important lessons for all.

The Penrose Cancer Center. a member of Penrose-St. Francis Health Services, revived the Penrose Cancer Seminars after a 16 year hiatus, and in September, 1993, the Penrose Cancer Conference debuted. In the tradition of the earlier conferences, the 1993 conference was again held in the Litue Theater of the Broadmoor Hotel. Conference participants • comprised of pathologists, radiologists and clinicians • focused their diagnostic skills on the difficult diagnoses of breast and ovarian neoplasms. A total of 91 participants attended. In 1994, 1995 and 1996 the number of attendees has increased.

Today, the Penrose Cancer Center is pleased and proud to continue the tradition of excellence in clinical education as an expression of its mission to manage the most complex malignancies and to assist in the eradication of this disease.

Previous Penrose Cancer Conference Topics

1949 Miscellaneous Tumors AP Stout, LV Ackerman

1950 Bone Tumors PJ Hodes, LV Ackerman

1951 Thoracic Tumors AP Stout, PJ Hodes, LH Garland

1952 Gastric Tumors LV Ackerman, LG Rigler

1953 Tumors of the Small Intestine PC Swenson, WA Meissner

1954 Tumors of the Large Bowel R Golden, RC Hom, Jr.

1955 Tumors of the Urinary Tract FJ Hodges, FK Mostofi

1956 Intracranial Tumors EP Pendergrass, JW Kemohan

1957 Cancer in Children EBD Neuhauser, BH Landing

1958 Bone Tumors PJ Hodes, LV Ackerman

1959 Tumors of Soft Tissues PJ Hodes, AP Stout

1960 Thoracic Tumors B Felson, R Lattes

1961 Gastric Tumors R Schalzki, WA Meissner

1962 Intracranial Tumors JM Taveras, HM Zimmerman

1963 Tumors of the Small and Large S Welin, B Castleman Intestines

1964 Tumors of the Urinary Tract JA Evans, LM Franks

1965 Malignant Tumors in Children JA Kirkpatrick, JM Kissane

1966 Malignant Tumors of the HZ Mellins, H Rappaport Lymphoid Structures

1967 Bone Tumors J Edeiken, HJ Spjut

1968 Thoracic Tumors M Viamonte, Jr., AA Llebow

1969 Gastrointestinal Tumors RD Moseley, Jr., MH McGavran

1970 Tumors of the Central Nervous HO Peterson, LJ Rubinstein System

1971 Tumors of the Urinary Tract AF Lalli, WC Bauer

1972 Pediatric Tumors J.C. Dumbar, H.S. Rosenberg

1974 Neoplastic and Non Neoplastic N Goodman. M Kuschner Diseases of the Chest

1975 Diseases of the Hepato-biliary A Moss, T Kent and Pancreatic Ducts, Ampulla of Vater and Duodenum

1976 Breast Lesions LM Kalisher, RW McDivitt

1977 Diseases of the S Wallace, R Hartsock, DAG Galton Reticuloendothelial System

1993 Breast Cancer and Ovarian 8 Eklund, 8 Gosink, PP Rosen, R Scully Cancer

1994 Soft Tissue Sarcomas RL Kempson, SW Weiss, MS Jochelson, CA Forscher

1995 Prostate and Testicular Cancer Jl Epstein, RD McLeary, JA Smith, Jr., TM Ulbright, AT Stavros

1996 Head and Neck Tumors S Mills, A Mancuso, R Weber

1997 rumors of the Chest TV Colby, SS Sagel, LP Faber

Penrose Cancer Conference 1997

Thomas V. Colby. MD Surgical Pathology Mayo Clinic Scottsdale Scottsdale. Arizona

L. Penfield Faber, MD Rush University Professor of Surgery Chicago, Illinois

Stuart S. Sagel, MD

Guest Discussants

Malllncllrodt Institute of Radiology Washington University Medical Center St. Louis, Missouri

P. Terrence O'Rou!Xe, MD. Chairman Medical Director. Penrose Cancer Center Penrose-St. Francis Health Services

Cos G. Sciotto, MD Pathology Department Penrose-St. Francis Health Services

Russ Lee, MD Colorado Springs Pulmonary Consultants, PC 25 East Jackson Street, Suite 202 Colorado Springs, Colorado 80907

Jon Snider, MD Radiology Departmenl Penrose-St. Francis Health Services

James R. Stewart. MD Cardiovascular Surgeons of Colorado Springs, PC 25 East Jackson Street, Suite 1 04 Colorado Springs, Colorado 80907

Ted T. Lewis, MD Director of Medical Education Penrose-St. Francis Health Services

Daniel Mayes, MD Pathology Department Penrose-St. Francis Health Services

Kay Petras, MBA Director, Penrose Cancer Center Penrose-St. Francis Health Services

Unda Gardner Administrative Assistant Penrose Cancer Center Penrose-St. Francis Health Services

Nancetta Williams, MBA Continuing Medical Education Coordinator Penrose-St. Francis Health Services


Committee

James Anderson, MD

Mitchell Bitter, MD


Cardiovascular Surgeons of Colorado Springs

-- .......... -~ .. - ...... ___ ··- ··-·-·· University of Colorado Health Sciences Center

Case Contributors

Colorado Springs, Colorado

Denver, Colorado

........ " ··-· ...... ,...... ... . ........................ . ......... ,., ,. _____ ............... ........ . .... _, ..... - ..................... __ ... _ .. _____ _ James Brown, MD University of Colorado Health Denver, Colorado

Sciences Center

........... wi.liiafii"cilamlieiS:·Wio- ............ coio'faCio" siiri'iiii5"suriiicai··· · ···· · ...... .. c.oi.oraC!o siiiiniis:· Associates Colorado

"'Raggio coitiY', i.lo· . .. ..... "F>oudre .. vai'ley .. Hosii'ii'ai ..... .. ...... F=i: coiiins·:·colora<io -·-· ..... .

Mary Cori<ill, MD

Steve Cullen, MD

Donald L. Dawson, MD

Memorial Hospital Colorado Springs, Color.ado

--· .......... ·- _ .. ___ ....... ........... ' +··-~-········ ·-' --· ......... . Penrose-St. Francis Health Colorado Springs, Services, Department of Colorado Pathology

.. - ·--··vr;i-c-e-oeP'inio~'Mo···-.... --.... coioiiid'Ci"siiiiilii's"i=i'iiaiiil'F>a.itiie·iS· ··-co.iiliii<io ··sil~fi9ii·.···· .. ············· Colorado

................ :··-ca'f;y-oiiiori:-r;;o······-.. --·-·-ri;ren;-o~ai'FfospiiaT--·---···-·-·--·-c:aioracio .. siliiriQ5·: ................. .. Colorado

.................. si·e:va··Ei'iason: .. Mo ..................... si:Tilii'iS''iJii.ivers~'Y-H'osliiiarscu-·-scr<>-urs:-MissoUii ____ ... _ .. Care

......... · Gaitil"Ei19ilini{ M'D ·· .... · · i''ii'liCire'Va.iieY'''Hoiiiiiai" ......... ..... ....... .. ;:(·caiii'iis~·colii.iiiiio-·--·-·

··-····-·· .. :iotii1'Re9siroiii:"MD--···--i'enros&:5t:-;:r&iicis.Healiii .................... coioF.i'do"silrinii's·:··· ............... . Services, Department of Colorado Pathology

- Ja·iii-es Hopfentiec·li:Mo · .. ... Uiiheraii .. MeCiiC'ai ce'ii'ier ....................... ·wileaiR'id'9e:"cCii.oi-'aCiil

Wi111am-E. Hiiiler, Mo ................. uiiiversi'iiof'coioiii'ci'o'Reaiiil ___ .......... oeiive·;:: .. coio'iacia ................... . Sciences Center

David Hurrma·ii·.-·M'o -·- ·-Rock'i.Moliiiiaiii-caiicer ......... --.. -.. -·coioiiido-sP'riiiiis:-·-·- ...... .. Specialists Colorado

Barry Lawshe, MD


Penrose-St. Francis Health Services, Department of Pathology

Case Contributors


................ ii.aronTo'ii!J": iiio""" ···· · ......... s1: Mar;:•;;· Hos.iiiia'f' .... · · ............... · ·· ·c;rS:nCi· :JiJii.clio·ii·;· Ciiio.iS:Cio·

·Wiiwa·;n··M'ala_ti_ra·: ·iiio·-·-·-.. -· .. -c:o.ioia!io-si'iiii95'H.eiilii1-i>art.neiS ..... coiora'do-si'rinlis:-·-·-.. --.... Colorado

..... ·· ·· ·Maik·M·: .. Man9a·iic;·:·Mo ................ c.oiii'm'bia .. R'iiili'ii'iiai"Nieeiicai"ceililii .. i=oii"Myers: i=iii'iiCia .... ........ . Southwest Florida

.. ........... · David'' Martz::· t.:io ............ R'ii'ciiY'·r:.:;c;i:iilta·i·ii .. ca·;;·c:er ......................... coiora'iio'si'rinils·:·... . .. ·· .. Specialists Colorado

........ _oari-NiaY'es: ·Mo ............... ·--·-p-;;-n;:c;s-8~si~f'raiicis_H.eaiiil·-·-··-·-·-coiora'das_,i_riiiils~·-·-·-·-·-·

Services, Department of Colorado Pathology

5ona'iii Mcclure, MD siifriTJosep·ii-HospiiaT_____ -·--·--.. i'ienve"i'; cOiCirado

·'Gene Mooie, Mo-- · ... r>eiirose-sCF'i'iiliCis'Rii'aiiti ................... cii'foraCio.siliin'9s:·········· .. ······

Joel S. Morris, MD

John Newcomer, MD

Eileen Nobles, MD

Services, Department of Colorado Pathology

Memorial Hospital

Colorado Springs Pulm-onary Consultants

Penrose-St. Francis Health Services, Department of Pathology


Colorado· Springs, Colorado


David Paiz, t.i'b ....... _ · - - si:'MaiY•5"H'ci5i>iial .. ---·--·-·--·-·-·-Grand :iuii.ciion, colorado

curiis .. J. Pink.' MD -· ................... Memo.iiii'I.HosPitar ...... -......... .... ... .... coloraCio .. siiiiii9s:···· ............ . Colorado

·-jiiiin · 'R'ii'iitioilo:· Nio····· ......... c.iiidi.iivasci:iiii si:irii'tioiis or .... ··- ··· ··· ··cii'ioraCio·s·iiii'ii95~ .................. .

Robert Sayre, MD

Colorado Springs Colorado

Rocky Mountain Cancer Specialists

·-·-·-·--·· . ····-·-·-·-···· .. ·-····-·----Colorado Springs, Colorado

.... . ............. ~ . . -· -·--···.. ........................ . ... ~ .............................. -... .. . ....... ,_ .............. . Larry Seidenstein, MD Columbia Regional Medical Center Fort Myers, Florida

Southwest Florida ............. ··riat ·;;,. stiati; .. Mo .......... .......... car'i''f''fiaY:iie'ii"vx·r:.:;·e-aicar·c:·;;·;;ie·r:··p·ii·o·eiilx:··A.·;i zona··-······ .......... .


Case Contributors

Daniel Slagel, MD St. Mary's Hospital Grand Junction, Colorado

· - .. J.on snider. MD ...... ·· · · F>enrose:·scf'riii1ci5 Hea'iii1 ................. c'Oiorad'o-sP'ri'ii'Q'5~----·-·-

services. Department of Colorado Radiology

................ 'James··siii:Waii:''MB ..................... caiiiiovasc·liiar .. siii'ii'eoiis"of' ................ coiora·cio.siir1nii's:···-·------·· Colorado Springs Colorado

............. i>aiiic'k-s'to·uciVio···-·-·-·····-·-··Memoriiii'iiosiiitaT"·-·-·--···---·-······· .. c:·c;lora<io.siiiii195~· .. ····-·· ...... Colorado

.. ........... oaiiiiiT'slii'iiva·;;·: .. M'D' ................... c.Hilicai"L'iiiioraioiY·ar!ii'ii"elack' ...... "kaii'ia"Cily·:soii'ifi"oaiioia" Hills

................. Robert· ·vamii'm~·r;;;·o ................... co·iorailo'siiiiiiiis·fi·Lii'rii'O'iiii'iY .............. coiorad'as·p-;ii1ii's:··-··---·-.. -· Consultants Colorado

--··· ·R':·oiiin'Y"Wet>-e·;:: 'Mo··-·----·--irireCifalis·o·is-i;;;S'e·s ·iieciaTiSis·· ... -..... coiorailo-sPiifi9s:--·-·-· · ........ Colorado

_ ......... ciiii.rlii·;;··ziiln: .. r;;;D·· .......................... R'ii'c'ky·r;;;o;;n\iii'ii.ca·n·c:e-;:--········ ............... coiiiiid'o .. siiiiiiiis.---........ .. Specialists Colorado

Penrose Cancer Conf~rence 1997

Case contributed by: Russ l ee, MD: Robert Varnum, MD; and Gos G. Sciotto, MD Penrose-St: Francis Health Services, Colorado Springs, Colorado

Clinical Presentation:

Case #1

2.4 year old female, 23 wee.ks pregnant, pFesented with dyspnea and cough. Her dyspnea began approximately five months prior to admission. At that time she also complained of fevers, purulent sputum and malaise. She initially improved with bronchodilators and antibiotics. Her dyspnea began to worsen and at the time of admission she noted dyspnea with minimal activity. She had no other significant paslmedical history. This was her second pregnancy.

Physical exam: Mild respiratory distress with cyanosis. RR 25 Chest -"vague" basilar crackles. Left upper extremity edema and an indurated, warm, tender left supraclavicular lymph node which increased in size afier admission.

CXR: Dense, reticular infiltrate throughout all lung fields.

Specimens: Open lung biopsy was carried out after a transbronchial biopsy was O'On-diagnostic.

Gross Pathology:

A 3.2 x 1.2 x0.9 em wedge biopsy of the right lung was received. The pleural surface had severa1 .0.2 em diameter cysts-bullae. On section similar 0.1 to 0.3 em cysts surrounded by fibrous tissue were observed. There was no gross area of consolidation.

Submitted Material:

Portion of lung.

Penrose Cancer Confe-rence 1997

Case conlnbYitd by: Russ lee, MD; R- Varnum. MD: and ~ G. Sclollo. MD Penrose·St. Frane•s Health Serv1ces. Calotado Spnngs, Colorado

Case #1

Figure 1: Chest X-ray

Penrose Cancer Confe(ence 1997

Cas• conlrrbulod by' Ruu Lee. MD, Robert Varnum. MD. and Cos G. Sc10:to, MD Penros.,.St Franc11 Hllath Semces. Co-torado Springs. Cdo:ado

Case #1

Figure 2: Chest CT

Penrose Cancer Confer~e 1997

Case #2

C&se cootnbuled by: Russ Lee, MD; Wi16am M411abre, MD; Barry L.aw.~e. MD and Robert Sayre, MD Penrou·St. Francis Heahh Services, Colorado Springs, Colorado

Clinical Presentation: 35 year old male presented with chest pain and dyspnea. He had been admitted with chest pain three and six months prior to this admission. Evaluation with an echocardiogram documented a pericardia! effusion and mild to moderate pulmonary valve insufficiency. Lab evaluation showed a normal Westergren ESR and an ANA that showed a speckled pattern at 1:64.

His symptoms improved with prednisone and his clinical diagnosis each time was pericarditis.

His dyspnea recurred one month prior to admission and gradually worsened to the point of being short of breath with minimal exertion. He complained of nonpleuritic chest pain for one week prior to admission that occasionally worsened with movement. He had hemoptysis (up to one cup per day) for one and 1/2 weeks prior to admission. He had gained 40 pouds while on prednisone. He denied fevers, chills or night sweats.

Physical examination: abefrile, RR 20, P92, BP 144/85

Dullness and decreased breath sounds at the left posterior chest. Otherwise unremarkable •

Lab: Hb 10.5 g/dl; WBC 9.6, with 75 segs; 16 lymphs, 8 monos, and 2 eos; platelet count- 547,000; LDH 494 u/1; SGOT 36 u/1

CXR: left sided pleural effusion and right sided nodular infiltrates.

CT chest: bilateral nodular densities (with indistinct borders) with pleural and pericardia! fluid.

Patient underwent open lung and pericardia! biopsies.

Gross Pathology: Two portions of pericardium 3.2 x 1.0 x x0.4 em and 2.0 x 1.2 x 0.4 em were submitted. One surface was smooth and glistening, the opposite was rough, irregular and friable. In addition to the pericardium there was a wedge biopsy of pink uniform left lung, 5.0 x 3.2 x 1.5 em

Submitted Material: Portion of pericardium and portion of lung.


Case #2

Case contributed by; Russ Loe, MO. Wolllam Malabre, MD; Barry Lawohe, MD and Rebert Sayre MD Penrose-St Fr~mcl& Health Se-N!ces, Colorado Springs, Colora.do

Figure 1;

Chest X-ray


Case #3

Case contribuled by: Daniel Slagel. MD; Aaron Long, MD; David Petz, MD a.nd Richard Funo.n, MD St. Mar{a Hospital, Grand Juncllon, Colorado


50 year old ski instructor who complained of dyspnea that was more noticeable while walking than skiing. He also complained of a non-productive cough. He smokes 1 1/2 packs of cigarettes per day.

Occupational exposure (summer) to concrete dust and muriatic acid.

Family history:

Labs: Hct-55

Father- died from a "lung problem" Brother -myelofibrosis

WBC -7,000

Platelet count - 86,000

CXR and CT Scan; diffuse, bilateral interstitial abnormality

A thoracoscopic lung biopsy was performed.

Gross Pathology;

Two biopsies of the right lobe were submitted. The right upper lobe biopsy was 3.0 x 1.5 x 1.0 em, the right lower lobe was 2.5 x 1.5 x 1.0 em. They contained a line of metal clips: The pleural surface had a mosaic tan to red pattern. The lung had focal firmness due to nodules and microcystic spaces were noted.

Submitted Material:

Lung biopsy.

Penrose ·Cancer ConiE!rencE! 1997

Case #3

Case contti6u!ed by. Daniel Slagel, MD, Aaron Long, MD, David Pat.z. MD and Rlctulrd Fulton, MD St. Mary's Hospital, Grand Junction, Colorado

Figure 1:

Chest X-ray

-

~ .. ·•

Figure 2:

Chest CT


Case#4

Caoe conlnbuted by Sieve Cullen. MD; Pat Stout. MD. Curtis J. Pink. MD and Joel S. Morris, MD Memorial Hospital, C- Springs, Cd«ado


A 59 year old Asian male with a history of tuberculosis presented with shortness of breath. He had a past history of sinusitis, allergies and chronic asthma. Mild pulmonary hypertension was found.

CXR and CT scan demonstrated bilateral interstitial lung disease. Bronchoscopy showed bilateral endobronchial nodules. A left thorcoscopy and wedge resection of the left upper lobe was performed. Several pleural adhesions were noted. Biopsies were taken from the lingula portion of the left upper lobe and superior segment of the left lower lobe.

Gross Pathology:

The left upper lobe wedge biopsy was 6.0 x 3.5 x 1.3 em pink-tan, partially anthracotic lung with vaguely nodular appearance. The left lower lobe was 6.5 x 1.5 x 1.4 em wedge potion of pink-tan lung with some gray nodules up to 0.2 em.

Submitted Material:

Portions of lung biopsies.

Penrose Cancer Conference 19:97

Case #4

Case contribU!ed by- Steve Cullen. MO; Pat Stout, MD; Cu~is J Pink. MO and Joel S. Morris, MD Menlorial Hosprtal, COlorado Springs, Colorado

Figure 1:

Chest X-ray, 1988

Figure 2:

Chest X-ray, 1996



Case contributed by: James Hopfenbeck, MO l utheran Medfcal Cenlor, WhutRidgt, Cok>rado

Case #5

61 year old male found to have bilatera l, small pulmonary nodules in 1986. Follow up CXRs showed an increase in size followed by regression of the nodules.

There was a subsequent increase in size of the nodules accompanied by symptoms of weight loss, fatigue, ptosis and lower extremity parasthesias, the clinical diagnosis was •tumor vs. Wegener's Granulomatosis".

Lab evaluations: Normal renal function, sedimentation rate and negative antineutrophil cytoplasmic autoantibodies (ANCA).

A thoracoscopic biopsy was performed.

Gross Pathology:

Several biopsies from the left lung were received. Two of the larger ones were:

one from the left lower lobe- a 4.0 gm, 4.0 x 3.0 x 1.0 em wedge and

the other from the left upper lobe- also a 4.0 gm biopsy.

The pleural surface was glistening pink red with anthracotic pigmentation. Sectioning demonstrated several 0.2 to 0.6 em tan nodules surrounded by spongy red brown parenchyma.

Submitted Material:

Lung biopsy.

Figure2:

Chest CT

12/92

case oon~1bUted by- James Hopfenbeck. MD ltAheran Medical Center. WheatRKige. Colonldo

Figure 1:

Chest X-ray

12/92

Pe.nrose Cancer C-onference 1997

Case #5


Case eonlni>Uiod by· James Hopfenbeck, MD lutheran Medical Center, WheatRidge, Colo<a<lb

Case #5

Figure 3: Chest X-ray 8/93


Case oontrii>Utad by: Mark M. Mangano, MD and larry Seldensteill. MD Columbia Regional Medical Ctnter Sou1l!wul Aori<la, Fofl Myers. Florida


Case #6

71 year old male with a history of diabetes mellitus presented with shortness of breath and a 9 pound weight loss. There was a remote history of tobacco use and he drank two to three beers per day.

Radiological evaluation revealed a large hilar mass

Following a Bronchoscopic biopsy a pneumonectory was performed.

Gross Pathology:

A 31 9 gm, 27.5 x 11 .5 x 4.5 em right pneumonectomy was received. The pleural surface was unremarkable. In the hilum several enlarged nodes are noted. On section the hilum contained a 2.5 x 2.0 x 2.0 em soft and tan mass near the bronchial resection margins. The lesion surrounded the main bronchus but did not invade it. The pleura was not involved.

Submitted Material:

Portion of the hilar mass


Case c;;onlnbu1ed by: Mark M. Mangano, MD and lany Setdenstein, MO C<Jiumboa RegiOOal Medlcal Center-- Florida Fort Myers. Floridll

Case #6


Figure 2: Chest CT

Penrose. Cancer Conference 1997

Case contributed by: Mark M. Mangano, MD and Larry -Sek1enstein, MD Columbia Regional Med:cal Center Southwest Florida, Fort Myers, Florida

Figure 3: Chest CT

Case #6

Penrose Cancer Conferenc-e 1997

Case#7

Case contributed by: James T. Anderson, MD; Donald L. Dawson, MD; Cos G. Sclotto, MD and Jon Snider, MD P~Wose-St. Francis Health SeNices, Colotado Springs, Colorado


51 year old businessman was evaluated for weight loss of about 35 pounds, polyuria and thirst. Diabetes was documented with blood sugar up to 450 mg/dl.

Patient had a 75-pack-year history of smoking. His only other complaint was left lower neck and shoulder pain, and symmetrical pain of distal radiuses and tibias; these he had for several years.

On a routine CXR a left infrahilar mass was found. CT scans showed 5-6 em left hilar mass, no additional lung lesions and a normal abdomen and head study.

A bone scan was negative.

A left lower lobe thoracotomy was performed.

Gross Pathology:

A left lower lobe, 15 x 11 x 10 em, contained a 5.5 em gray-white, soft, focally necrotic mass which extended to the pleura. Surrounding the mass noted was atelectatic lung tissue and small blebs. Hilar nodes were anthracotic.

Submitted Material:

Portion of the left lobe mass.


Case #7

Ca•e contro~uted by, Jemes T Anderson, MD; Donald ~. Dawson. MD; Cos G. Scocl!o, MD and Jon Snider, MD Pe-nrose-St. Francis Heatth SeN1Ces. Colorado Springs, Co!orado


Ftgure 2: Chest CT


Case oonlributed by: Donald McClure, MD St. Joseph Hospllal, Denver, Colorado


Case #8

A 46 year old female presented with vaginal bleeding. There were no complaints of shortness of breath, cough, sputum production or chest pain. She underwent a D&C under general anesthesia. The intubation was difficult and post-operatively she complained of neck and chest pain.

CXR revealed a right lower lobe mass.

Gross Pathology:

The sample was a 62 gm, 6.1 x 6.0 x 3.4 em right lung mass which was ovoid, fleshy pink and spongi form. It lacked a distinct capsule. On section it was spongy, purple, vascular and pitted; it resembled placental parenchyma.

Submitted Material: Lung mass

Figure 2: Chest X-ray,

Lateral

Case contributed by: Donald McClure. MD Sl Joseph Hospial Denver, Colorado


Case #8

Figure 1:

Chest X-ray, PA


Case #9

Case contributed by: stew Eiason, MD St. Louis University Hospital, SLU Core, St. Louis, Missouri


70 year old male with a two year history of intermittent night sweats. He denied any other complaints.

Routine CXR revealed a question of a mass vs. cardiomegaly. A chest CT scan showed a mediastinal mass possibly arising from the thymus.

At surgery a 23x18x12 em "fatty mediastinal tumor" was resected without significant adhesions.

Gross Pathology:

Specimen consisted of a large, fatty, 23 x 18 x 12 em portion of thymic mass. On section it was uniform soft yellow and fatty.

Submitted Material:

Portion of mediastinal tumor.


Case #9

Case c;onlul>u1ed by. S1eve Eliason, MD St LOUIS Un<ve;s<y Hospilal. SLU C.re. Sl. LOuiS. Mls><>uri

Figure 1:

Chest X-ray

I

Figure 2:

Chest CT

Penrose Cincer Confettnce 1887

Case #10

Caso contributed by: M~ch Bitter, MD; WUIIam e. Huffer, MD and James Brown, MD University of Colorado Heallh Sciences Center, Donver, Colorado

and Jon Snide<, MD and Russ Lee. MD

Penrose-St. Francis Health SeNices, Colotado Springs, Colorado


A 35 year old male presented with a three day history of malaise, weakness and cough productive of pinkish sputum. He had become more short of breath the day of admission. He also complained of months of dysphagia with solid food associated with reflux and regurgitation. This had transiently improved with Prilosec. He had an episode of melena prior to admission. He had lost an unknown amount of weight He had a history of mitral valve prolapse.

He had undergone a right pneumonectomy at age 12, the pathology showed congenital cystic disease, hamartomatous bronchial formation. He had undergone tracheal laser treatments for an unknown lesion 14 years prior to admission.

The physical exam was remarkable tor:

normal vital signs

pallor of the conjunctival membranes

well healed right thoractomy scar decreased right sided breath sounds, left lower lobe rhonchi

2/6 systolic murmur at left sternal border without radiation

Labs: Hb 10.6 g/dl; MCV 64; Fe 8; TIBC 285; Fe% saturation 3; Ferritin 13

CXR: left lower- lower lobe infiltrate, s/p right pneumonectomy

Chest CT scan: large mediastinal mass and left lower lobe consolidation with air bronchograms

The patient subsequently underwent a thoracotomy.

Gross Pathology:

Several samples were received at the time of resection. The main sample was a 14.5 x 13.0 x 7.8 em aggregate of fibrous and adipose tissue containing portions of pleura A portion of esophagus with fatty mass up to 18.3 x 11.5 x 6.7 em was submitted, a 8.8 x 7.8 x 6.2 em mass protruded from the esophagus. Several submucosal nodules, 2.0 to 4.0 em were noted in the wall of the esophagus. A separate 2. 7 x 1.5 x 0.9 em fatty aggregate was designated as "mediastinal tumor".

Submitted Material:

Soft tissue around esophagus.

Penrose Cancer Conferenc.e 1997

Case #10

CasQ conhibuled by. Mltoh Siner, MO; Wilham E. Hutter, MO and James Brown. MO Uni'lershy or Colorado Health Sciences CQnter, Oenvet. Colorado

and Jon Snlder. MD a<1<1 Russ l eo, MD

Peorose·St Francis Health Services, Colorado Springs, Co1orado-

Figure 1:

Esophagram


Case #10

Case contributed by Mitch Blner, MD; Wilham E.. Huffer, MO and James Brown, MD Uni\•er$l,y of Colorado Health Sciences Cenler, Oenve:, Colorado

and Jon Snider. MD OJld Russ Lee. MD

Pem0$0-St. Francis HeaHil Services, Colorado Springs, Cototado

Figure 2: Chest CT

Penr0$e cancer Conference 19.97

Case #10

Case co-nlriblfled by Milch Bitter, MD; W illiillll E. Huffer, MD and James Brown, MO Unwors•ly of Cotorado Health Sciences Center, Denve~. Colo~do

and Jon Snider, MD and Rust Lee MD

PetYos .. st. Frant<S H•alth Services. Colorado Spnngs. Colorado

Figure 3:

Chest CT

Penrose Cancer Conrerenee 1997

Case #11

Cue contributed by: James Stewart, MD, Russ Lee, MD; John Hegstrom, MD and Dan Mayes, MD Penrose-St. Francis Health SeMc:es, Colorado Springs, Colorado


78 year old male presents with a 2 month history of shortness of breath, cough, occasional yellow sputum and a 15 pound weight loss. A CXR showed a large left pleural effusion and he had mild hypoxemia (room air 96-88% Sa02). Physical exam was remarkable for dullness to percussion and decreased breath sounds at the lower 2/3 of the left lung.

A thoracentesis was performed.

A sputum cytology was suspicious for malignancy and cytology of the pleural fluid was positive for malignancy.

CXR (post-thoracentesis)showed a pneunothorax vs. trapped lung vs. large bullae

A thoracoscopy was performed with resection of giant bullae, wedge resection of the left lower lobe and talc pleurodesis.

Patient was found to have two giant apical bullae,

Multiple 1-2 mm pleural implants and consolidation of the left lower lobe with "obvious malignant tumor"

Gross Pathology:

Several specimen were received including multiple 2-5 mm yellow to light tan pleural biopsies, a 4.5 x 2.0 x 2.0 em wedge resection of left lower lobe and a 10 em diameter, 1 mm thick, smooth walled cystic structure from the left lung.

Submitted Material:

Portion of peripheral lung.


Case #11

Ca•e oonulbuled by: James Slewl!rt, MD, Russ Lee, MD; John Heg&trom, MD and Dan Mayes, MO Penrose-St F1oncl& Health Services. Colorado Sprlnos. Colorado

Figure 1:

Chest X-ray, 1197

Figure 2:

Chest X-ray, 2197

7

Penrose Cancer Conrerenc;e 1997

Case #11

Case contribtlled by James Stewart. MD, Russ Lee, MD: John H~gstrom. MD and Dan Mayes. MO Penros1!·SL Francis Health Services. Colorado Springs, Colorado ·


Case #12

Case comrib<Jted by. llat A Shah, MD; Leslie A, Wl>eeler, RN and ~rna S. Ga<1i, MD Ca~ T. Hay<len VAMC, Phoenix, Arizona


A 63 year o ld male with a 46-pack-year history of smoking and alcohol use was admitted in February 1994 because of a 40 pound weight loss over a period of six months. In the past month he had experienced increasing cough with yellow sputum, left pleuritic pain and shortness of breath. There is no history of asbestos exposure and he denied fever, chills and hemoptysis. Decreased breath sounds on the left were found on physician exam.

CT scan demonstrated a 14 em anterior mediastinal mass displacing the left lung. There was no cervical or axillary lymphadenopathy. A large left sided pleural effusion was present. A CT guided FNA was done. He had a rapid deterioration of his pulmonary status. Patient expired two days after the biopsy procedure and seven months after the onset of symptoms.

Gross Pathology:

A 1.5 li ter left sided pleural effusion and a 15 x 11 x 10 em gray-white, soft left apical pleural mass was found at the time of autopsy. The tumor extended over the left pleural cavity and involved both the visceral and parietal pleurae. Focal involvement of the diaphram and mediastinum were found. The left lung was atelectatic with encasement by a 3 em thick tumor. Hilar and mediastinal node metastases were found along with bilateral bronchopneumonia

Submitted Material:

Peripheral lung tissue from autopsy.

Penrose Cancer Conf:erenc.e '1997

Case #12

Case ecniNbuled by: Ita< A. Shah, MO. Leslie A. Wheeler. RN ""'! Osama S Gani. MD Ca~ T VAMC, Pho""'x Arizooa

Figure 1:

Chest X-ray

Penrose C1ncer Confertnce 1897

Case eonlribuled by: Ra,ggio Colby, MD and Garth Englund, MD Poudre Valley Hospital, Fl. Collins. Colorado


Case #13

Asymptomatic 73 year old female underwent a routine pre-op CXR for knee replacement.

CXR revealed a 6x7 em mass abutting the posterior pleural surface of the left upper lobe. No previous studies were available for comparison.

On CT scan a 4x5 em mass adjacent to the descending thoracic aorta at the level of the aortic arch was found. No calcifications were present. The mass was "smooth bordered". Small focal scar was noted at the left lower lobe. No other abnormalities were seen.

A left lower lobectomy was performed. The mass was found attached to the lung but not involving it.

Gross Pathology:

The tumor mass was 40 gm, 5.0 x 5.0 x 3.5 em with a tan-red to dark red surface attached to 6.0 x 3.0 x 1.0 portion of membranous tissue.

On section it was rubbery gray-tan to focally gray-white. A 5.0 x 2.5 x 1.5 em red wedge-shaped porti!Jn of lung was also received, it lacked any lesions.

Submitted Material:

Pleural nodule.

Figure2·

ChestC

Penrose. Cancer Conference 1997

Case eonrnOO!ed bY' Ragg.o Colby, MD ond Garth Englund, MD PoUdre VaDey Hospital, FL Collins. Colorado

Case #13


+ PENROSE-ST. FRANCIS HEALTH SERVICES Centura Health,.

August 13, 1997

Juan Ro s .ai, M. D. Department o f Patho logy Memorial Sloan-Ke tteri ng 1275 York Avenue New Xork, NY 10021

Dear o r. Roeai:

Department of Pathology Penrose Pa thology Group, P.C.

2215 N. Cascade Colorai:lo Springs, Colorado 80907 • 7699

(719) 776·5816 FAX: (719) 776·5584

M. Berlhrong, M.D. 0 . Franq uemont, M.O.

J, Hcgs,Lrom, M.D. T. Kirc.her, M.O. 0. Law-she, M.D.

D.C. Mayes, M.O. G. H. Moore. M.O. E. M. Nobles, M.D.

C.C. Sc:iotto, M.O., Ph.O. R. M. Sherwin, M.D.

Enclosed is the syl labus alo ng wi t !:\ a set of H&E slid es f or the upcoming Pe nrose Cancer Conference which wi ll be held on September 12, 1997, in Colo rado Spr ings. we a l l appreciate your will ingness to act as a n expe r t c onsult ant on t he cases. Ourinq the conference, we wi l l acknowledge your participation.

Thank you again for time and interest.

Si~0~ C. G. Sc i otto , M.D.

Enc losure

CGS/mh

}tum Uos<~i, MIJ Chai,.num. IJ'fkutmml of Pu1bol•g)

ymNJ Etmtr, Aluomi CIJair ;, Pdtbo/Agy

August 28, 1997

Cosimo Sciotto, M.D. Department of Pathology Peru-ose Pathology Group, P.C. 2215 N.Cascade Colorado Springs, CO 80907-7699

Dear Cosimo:

Thanks for sending me the slides and syllabus of the 1997 Pemose Cancer Confere nce. Enclosed please find my comments on the cases.

Best wishes for a successful seminar. Please give my best regards to Dr. Berthrong.

db Enclosure

Sincerely yours,

Juan Rosai. M.D.

IHt•marifll .\'/o(ln .. Krl/('''i n.~ Canr'er Cemer t27j ) ~Jr.l! l\nm11e. New Yt;rk. Neu• ,'lwk J0021

'li·lrplwte 212.6w.8•l"' • F,,x 212772.8pt

.... } . " -~ "

CASE 1: Very nice case of lymphangiomyomatosis. It ought to be HMB-45 positive.

CASE 2: I suspect this is a sarcoma and specifically an angiosarcoma, but I would need the stains to rule out mesothelioma and other alternatives. The prominent involvement of several large pulmonary vessels 1·eminded me of the following intriguing comment made by Averill Liebow in the discussion of Case 2 of the Penrose Cancer Seminar on Tumors of Lung and Mediastinum he ran on November 2, 1968, and which I attended:

"There are interesting conditions in the lungs, which I like to call "sarcomatosis," which seem to follow the branches of the pulmonary artery and also the bronchial tree. I don't know why, it is a very puzzling thing. We must have seven to ten of these. They seem to extend directly along the walls of these structures as if some stimulus were gradually convering the tissue to what is rapidly proliferating and quite clearly malignant."

These probably have nothing to do with the present case, but I have often wondered what he was referring to. Maybe Tom Colby knows.

CASE 3: Langerhans' cell histiocytosis (eosinophilic g1·anuloma)

CASE 4: I pass on this one.

CASE 5: 1 pass on this one, too.

CASE 6: I favor a large ceWanaplastic malignant lymphoma, but I need the stains.

CASE 7: Carcinoma. I think it is compatible with n lung primary, but it could also be thymic or metastatic from a distant source.

CASE 8: It looks like n vascular tumor of some sort. It if had been in the cerebellum I would have called it a hemangioblastoma. I also thought of a metastatic

uterine mesenchymal tumor(? endometrial stroma), but it just does not look like it.

CASE9: Very good example of atypical lipomatous tumor (sclerosing welldifferentiated liposarcoma)

CASE 10: This may be another atypical lipomatous tumor, but I cannot make that diagnosis on the basis of this single slide, which I would regard as compatible with lipoma or lipomatosis.

CASE 11: Adenocarcinoma with broncholonlveolar/Clara cell features. Beautiful nuclear true inclusions.

CASE 12: Anaplastic malignant tumor. I suspect it is a carcinoma, but one would need immunostains to categorize it properly.

CASE 13: Solitary fibrous tumor of plew·a. I wish that all the cases of this Seminar had been as easy as this one.

+ P.O. Box 7021 Colorado Spnncs. Color.ado 809ll Tcl719.776.5000

Centura Health~ PENROSE-ST. F RANCIS HEALTH SERVICES

9 Odober, 1997

Juan Rossi, M.D. Department of Pathology Memorial Sloan-Kettering Cancer Center 1275 Yorl\ Avenue New Yorl\, New Yorl\ 10021

Re: Penrose Cancer Conference 1997

-:r~ DearDr.~

Thank you for again for participating in the 1997 Penrose cancer Conference. The conference went very well.

Enclosed is a copy of information distributed at the Conference, which includes the discussant's. expert and participant diagnoses for the cases.

1 hope my edited comments regarding your diagnoses were acceptable.

Once again, thank you for your participation, we look forward to future input from you.

~"? ~"~

C. G. Sciotto, M.D.

Enclosure

CGS/n

Penrose Cancer Conference 1997 L. Penfield Faber, MD

DIFFERENTIAL DIAGNOSIS

• INTERSTITIAL LUNG DISEASE • SARCOIDOSIS • ASTHMA • HEMOSIDEROSIS • LYMPHANGIOLEIOMYOMATOSIS

Case 1

LAM CLINICAL PRESENTATION

• EXERTIONAL DYSPNEA • HEMOPTYSIS • PNEUMOTHORAX • CHYLOTHORAX • PREMENOPAUSAL WOMEN • DELAY IN DIAGNOSIS

Case 1


L. Penfield Faber, MD

LAM DIAGNOSIS

o CLINICAL AWARENESS • CT SCAN • PULMONARY FUNCTION

- FEV1 /FVC DECREASED -DIFFUSION DECREASED - Pa02 DECREASED

o TRANSBRONCHIAL LUNG BIOPSY • OPEN LUNG BIOPSY

Case 1

LAM THERAPY

• OOPHORECTOMY -VARIED RESPONSE

• TAMOXIFEN (ANTIESTROGEN) -VARIED RESPONSE - DO NOT RECOMMEND

• PROGESTERONE - ESTROGEN ANT AGONIST

Case 1


LAM PROGNOSIS

• SURVIVAL - 78% (25/32} 8.5 YRS.

• PROGRESSION VARIES

Case 1

'


Case #1

• Follow-up: -treated with tamoxifen, depo-provera, and radiation

to pelvic area

- patient moved to Florida


• DIAGNOSTIC CONSIDERATIONS -Age and sex: young woman

- Holes in the lung

- Spindle cell fascicles • ? Smooth musde • Cytologically peculiar • Vacuolated

Case#1


• HOLES IN THE LUNG -Emphysema

- Honeycombing

-Cystic diseases (Eg. BPD, CCAM, etc.)

-Cystic tumors (especially sarcomas)

- Lymphangioleiomyomatosis (LAM)

- Interstitial emphysema

- Overinflation of biopsy

-Others

Case#1

Penrose Cancar Conferen.ce 1997

Case #1

• SMOOTH MUSCLE IN THE LUNG - Normal: vessels, airways and alveolar ducts

- Scars with smooth muscle metaplasia

- Fibrosing interstitial pneumonias (UIP)

- Lymphangioleiomyomatosis (LAM)

- Hamartomas with smooth muscle

- Smooth muscle tumors (primary or metastatic) • Benign metastasizing leiomyoma (BML)

Penrose Cancer Conft rence 1997

Case #1

Submitting Pathologist's Diagnosis: Lymphangioleiomyomatosis

Dr. Colby's Diagnosis:

Lymphangioleiomyomatosis.


Case #1

• HMB-45 positive , actin positive, desmin positive

• Relationship to: - Tuberous sclerosis ( 1% women with TS have

LAM)

- Angiomyolipoma (HMB-45 positive)

- Clear cell tumor (HMB-45 positive)

Penrose Cencer Confetence 1997

Case #1 Expert consultants

Askin Lymphangiomyomatosis

Chan Lymphangiomyomatosis

Dail Lymphangioleiomyomatosis

Hammar Lymphangioleiomyomatosis - -·-·--··--··

Koss Lymphangioleiomyomatosis

Rosai A very nice case of ---·-··---J.Y-mphan.9.iQmyom"!tosis ·-,-- - -Travis Lymphangioleiomyomatosis

Weidner Lymphangioleiomyomatosis (LAM)

Yousem Lymphangioleiomyomatosis


Participant diagnoses Case #1

Lymphangioleiomyorratosis 19

• Lyrrphangiomyorratosis 2

Rbrom.~scular hyperplasia 1




• WEGENER'S • MIXED CONNECTIVE TISSUE

DISEASE • MESOTHELIOMA/EMBOLI • LUPUS ERYTHEMATOSIS

Case 2

RESPIRATORY LUPUS

• COUGH, FEVER • DYSPNEA • HEMOPTYSIS • PLEURITIS • PLEURAL EFFUSION (ANA) • PERICARDITIS (25%) • PULMONARY HYPERTENSION · ANEMIA

Case2

Penrose Cancer Conference!

L. Penfield Faber,

LUPUS DIAGNOSIS

• PLEURITIS • ANA 1 : 64 • PREDNISONE RESPONSE • HEMOPTYSIS • PULMONARY HYPERTENSION • RIGHT HEART FAILURE • ANEMIA

Case 2

LUPUS ETIOLOGY

• GENETIC • DRUG INDUCED • ENDOCRINE FACTORS • IMMUNE FACTORS

•

Case2



LUPUS PROGNOSIS

• 10 YEAR SURVIVAL- 79% • TREATMENT

- CORTICOSTEROIDS -IMMUNOSUPPRESSION

Case 2

MIXED CONNECTIVE TISSUE DISEASE

• ARTHRITIS, SEROSITIS, FEVER, MYOSITIS

• OVERLAPPING FEATURES -DERMATOMYOSITIS, SJOGREN'S - SLE

• PLEUROPULMONARY DISEASE • RESPONDS TO STEROIDS • PULMONARY HYPERTENSION • PULMONARY ARTERY

HYPERTROPHYNASCULITIS Case 2

•

Penrose Cancer Conference

L. Penfield Faber,

MESOTHELIOMA PERICARDIUM

• ASBESTOS EXPOSURE • HYPERPLASIA VS NEOPLASIA • RULE OUT METASTATIC CANCER • FATAL

Case 2

•

PenroS<O Cancer Conference 1997

Case#2

• Follow-up: - patient refused therapy

- was referred to hospice and expired

-an autopsy was refused

Penrose Cancer Confetenc::e: 1997

Case #2

Submitting Pathologist's Diagnosis:

Angiosarcoma


Metastatic angiosarcoma to the lung from the pericardium .

l

Pervose Cancer Confarence 1997

• CASE 2: KEY FEATURES -Intravascular malignancy

- ? Differentiation

- Carcinoma vs. sarcoma vs. other

Case#2


Case#2

• VASCULAR AND PERIVASCULAR MALIGNANCIES IN THE LUNG -Sarcomatoid/spindle cell carcinomas (1 o and 2°)

- Metastatic angiosarcoma

- Pulmonary artery sarcoma

- Kaposi 's sarcoma

-Other sarcomas

-Intravascular lymphomatosis

-[Organizing thromboembolic disease]

Pervose Cancer Conference 1997

Expert consultants Case#2

Askin Angiosarcoma -ch'a·;;·-·-----··-- intima·i··;,-ar:c<>·n;a--· .. -·-·-·----·---···-·----·-------oail Right pulmonary outflow tract sarcoma

Hammar

Koss

Rosai

Travis

Malignant neoplasm- diff Dx: angiosarcoma, renal cell carcinoma, neuro.endocrine ca. Epithelioid angiosarcoma

Suspect this is an angiosarcoma, R/0 mesothelioma & other alternativ.:::e,=s...,.---.,.,-Angiosarcoma, probably pericardia! or cardiac

W eidner Angiosarcoma, pulmonary artery sarcoma. ________ .. _carC!!.?_C angiosarcoma Yousem Angiosarcoma

Penrose C.ncer Conference 1997

Expert diagnosis Case #2

• Dr. Rosai - The prominent involvement of several large pulmonary

vessels reminded me of the following intriguing comment made by Averill Liebow in the discussion of Case 2 of the Penrose Cancer Seminar on Tumors of Lung and Mediastinum he ran on November 2, 1968, and which I attended:

- "There are interesting conditions in the lungs, which I like to call "sarcomatosis," which seem to follow the branches of the pulmonary artery and also the bronchial tree. I don't know why, it is a very puzzling thing. We must have seven to ten of these. They seem to extend directly along the walls of these structures as if some stimulus were gradually convering the tissue to what is rapidly roliferating arfd uite clear! malignant."

Penrose Cancer Conference Ht97

Participant diagnoses Angiosarcoma

Mesothelioma

Leiomyosarcoma ---·---·-· .. ·-····-·····-···-·····--· Lupus erythematosis

Epithelioid hemangioendothelioma

Possible lymphoma

Kaposis sarcoma

Angiosarcoma of the pericardium

•

Case #2

6 5

2

2

2

1

1

1




• BRONCHOPULMONARY ASPERGILLOSIS

• EOSINOPHILIC GRANULOMA • BRONCHOCENTRIC

GRANULOMATOSIS • DESQUAMATIVE INTERSTITIAL

PNEUMONIA

Case 3

BRONCHOCENTRIC GRANULOMATOSIS

• CLINICAL PRESENTATION - COUGH, DYSPNEA, HEMOPTYSIS -MALAISE, FEVER -BLOOD EOSINOPHILIA -ASTHMA

• TREATMENT - STEROIDS

Case 3


L. Penfield Faber, M\

EOSINOPHILIC GRANULOMA

• (HISTIOCYTOSIS X) • CLINICAL PRESENTATION

-COUGH, DYSPNEA -PNEUMOTHORAX -SMOKERS -VITAL CAPACITY DECREASED - ABNORMAL ROUTINE X-RAY -20% ASYMPTOMATIC

Case 3

EOSINOPHILIC GRANULOMA

• PROGNOSIS -RESOLVE, STABLE, PROGRESS -STEROIDS TREATMENT

Case 3

Penrose Cancer Conrorence 1997

Case#3

• Follow-up: - patient's father died of eosinophilic granuloma

- patient has done well after three months of steroid therapy with decreasing dyspnea

-subsequently he had an episode of DVT to~ lowed by dyspnea; he responded to anti-coagulation

-following the PE , he has been treated with steroids

-he is clinically stable


Case#3

• Dr. Slagel would like to know if there have been other reports of familial pulmonary histiocytosis X; is it prognostically important?

Penrose Canotr Conference 1997

Case#3

• APPROACH - Cellular micronodules

- Cytologic composition

- Clinical history: smoker


Case#3


Eosinophilic granuloma


• Pulmonary Langerhans cell histiocytosis (LCH).

• (Pulmonary histiocytosis X, pulmonary eosinophilic granuloma, pulmonary Langerhans cell granulomatosis)


Case#3

• PULMONARY LCH - Diagnosis (TBBx, BAL, OLBx)

• Support from immunohistochemistry (S100, CD1a)

-Management: Stop smoking(+/- steroids)

- Typically: Stellate nodules and cysts of pulmonary LCH

- Variants of pulmonary LCH

P•nrose Cancer Conference 1997

Case #3

• VARIANTS OF PULMONARY LANGERHANS CELL HISTIOCYTOSIS (LCH) - Solitary nodule/large nodules (2 em)

- Active: Nodules without cysts

- Healed pulmonary LCH

- Asymptomatic (radiologically apparent)

- Clinically occult (radiologically Inapparent)

- Question: How frequent is occult pulmonary LCH?

Penrose C•nc.er Conference 1997


Askin Chan Dail

Hammar Koss

Rosai Travis Weidner

Yousem

Langerhans' cell histiocytosis

Langerhans' cell histiocytosis Pulmonary Histiocytosis X

Langerhans' cell granulomatosis- PHX Langerhans' cell granulomatosis Langerhans' cell histiocytosis

Langerhans' cell histiocytosis Eosinophilic granuloma

Langerhans' cell histiocytosis

Pt ntose Cancer Conference 1997

Case#3 Participant diagnosis

Eosinophilic granuloma (Langerhans' 16 cell histiocytosis) Hypersensitivity pneumonitis and 2

e~Qhx~em~a----~--~----------~ Pulmonary hemosiderosis 1

Fibrosing allergic angiitis 1

Bronchocentric inflammatory process 1




• WEGENER'S GRANULOMATOSIS • EOSINOPHILIC PNEUMONIA • HYPERSENSITIVITY REACTION • ALLERGIC GRANULOMATOSIS

- (CHURG-STRAUSS SYNDROME)

Case 4

CHURG-STRAUSS SYNDROME

• CLINICAL PRESENTATION -ALLERGIC RHINITIS -SINUSITIS -ASTHMA -BLOOD EOSINOPHILIA -SKIN RASH

• VASCULITIS

• THERAPY -CORTICOSTEROIDS

• CYTOXAN Case 4

I

Penrose Cancer Conforence 1997

Case#4

• Follow-up: - Patient was treated with steroids and cytoxan (50

mg/day) - after 2 months of treatment he was feeling well, with

occassional sinus drainage and absence of pulmonary symptoms

- he can walk a mile or more without difficulty

- CXR (6 .30.97), four months after biopsy, was stable with a diffuse bilateral reticular pattern and associated hilar lymphadenopathy

Pervose CaneE!r Conference 1997

Case#4

• ISSUES - Lymphoproliferative versus inflammatory lung

disease

- If lymphoproliferative-subclassification ?

-Reactive: neoplastic

- Some cases remain indeterminate

- Management of the individual patient

Penro~ Cancer Conference 1997

• LYMPHOPROLIFERATIVE VERSUS INFLAMMATORY LUNG DISEASE

Case#4

- Lymphangitic (or discrete expansile nodules) versus diffuse polymorphous infiltrate

- Density and homogeneity of the infiltration

- Extent of fibrosislloss of architecture

- Clinical findings: SPEP, extrapulmonary disease, CLL, drug, rxn, collagen vascular disease

Penrose cancer Conference 1997

Submitting Patholgist's Diagnosis: Atypical Lymphoid infiltrates

Dr. Colby's Diagnosis: • Histologic diagnosis: Most consistent with

lymphoproliferative disorder (not DPB).

Case#4

• Molecular diagnosis: T cell lymphoma (T gamma receptor rearrangements)

Penrose Cancer Conference. 1997

Case #4

• PULMONARY DISEASE IN ADULT T CELL LEUKEMIA -Yoshioka et al. (Cancer 55:2491 , 1985):

Pulmonary infiltration with "chronic lung disease" prior to the diagnosis of adult T cell leukemia.

- Ono et al. (A J Hemato/30:86, 1989): Diffuse panbronchiolitis as a complication in patients with adult T cell leukemia; 3 of 43 cases, which is higher than the overall incidence of diffuse panbronchiolitis (DPB).

Penrose Cancer Conflfence 1997


Askin

Chan

Lymphoproliferative process (need IHC stud ies) Extrinsic allergic alveolitis

Dail Sm all lymphocytic lymphoma/MAL Toma vs ___ T -cell ·--~--::--=c--------

Ham mar Lymphoproliferalive Disease •........ ... - ------ - ·-·--

Kess Favor malignant lymphoma -- ·- ....... _ ..... ___ ,_.__,_ .............. -~ .... -··· ·-·-- -· ···--··· ........... _ Rosai Travis

Weidner

Pass on this one Lymphoproliferative disorder, favor NHL Possible Hodgkins, allergic bronchopulmonary aspergillosis, need more information for diag'""n"'o.:::.si"'s _ _ _ ___ _

Yousem Low grade lymphoma

3


Participant diagnoses Case#4

-P.:i19io.cenlric-iritlam·maTCirY"P"foces·s··----------------3---chronic eosinophilic pneumonia 3 -·---·-~·-·-·-·-·-·-·.,-·-·-·--··---·--·--·-·-···· ... ···· .. ······---·············-·-· Lymphoproliferative process 3 Malignant lymphoma 3

We-gener's granulomatosis 3 ExtrinsJc all erg i-c-a-:-lv_e_o-::1::-iti,.,.s---------=2-

Sarcoidosis 2 Angiocentric immunoproliferative leSio_ri ____ 1 __ Hypersensitivity pneumonitis 1 Inflammatory ps.eudotumor 1 Lymphoid inf iltrate 1




• WEGENER'S

• LOW GRADE LYMPHOMA

• WELL DIFFERENTIATED LYMPHOMA

• LYMPHOMATOID GRANULOMATOSIS

CaseS

LYMPHOMATOID GRANULOMATOSIS

• CLINICAL PRESENTATION -WAX AND WANE -COUGH, DYSPNEA, PAIN - CRANIAUPERIPHERAL

NEUROPATHY -SKIN RASH

-BONE LESIONS -SPECTRUM OF LYMPHOMA

Case 5

I

Penrose Cancer Conference ·

L. Penfield Faber, I

LYMPHOMATOID GRANULOMATOSIS

• PROGNOSIS POOR

• DEVELOP MALIGNANT LYMPHOMA

• TREATMENT -CORTICOSTEROIDS -CYTOXAN

-CHEMOTHERAPY

Case 5


Case #5

• CASE 5: ISSUES - Lymphoproliferative vs inflammatory

- If inflammatory-- how to classify?

- Criteria for L YG/angiocentric lymphoma

- Nodules, necrosis, angiitis, polymorphous lymphoid infiltrate

- Does this case fit the criteria?

- Distinction from low grade (MALT) lymphomas

- Long history; evidence of regression


Case #5

• Follow-up: - Patient was treated with chemotherapy and

developed neutropenia and serratia sepsis during his first course of therapy.

-He died of sepsis and multi-organ failure. -A post was not granted.

I


Submitting Pathologist's Diagnosis: lymphomatoid granulomatosis


Case #5

- Most consistent with lymphomatoid granulomatosis/angiocentric lymphoma.


Case#5

• EVOLUTION OF LYMPHOMATOID GRANULOMATOSIS - (Angiocentric lymphoma)

• 1970's--peculiar vasculitis; ? lymphoproliferative

• 1980's--lymphoproliferative disease - T cell proliferation

- Angiocentric immunoproliferative lesions (AIL)

• 1990's--lymphoproliferative disease - T cell rich EBV-driven B cell proliferation(? T cell rich

B cell lymphoma)

Petvose Cancer Conference 1997


--·· Askin

Chan

Dail

Hammar

Koss

Rosai

Travis

Weidner

Yousem

MALT lymphoma

Malignant lymphoma

Aspiration with benign lymphocytic and macrophage response Low grade AILIL YG

Lymphomatoid granulomatosis

Pass on this one, too

Small lymphocytic lymphoma

Pulmonary angiocentric immunoprolifertive lesion (AIL) Angiocentric lymphoma/L YG



Lymphoma 10 ~~---------~---~-~~--~~

Angiocentric immunoproliferative lesion 3

Infectious granuloma 2

Wegener's-like granulomatosis 1

Lymphoproliferative process 1

Lymphomatoid granulomatosis 1 -----------·-·--·--·-·-------.. ···-·---···-·······-··· ........ ______ , ....• DIP 1

Benign histiocytic lesion 1

Angiocentric lymphoma, low grade 1

.!




• METASTATIC CANCER • LUNG CANCER • LYMPHOMA

Case6

PULMONARY LYMPHOMA

• CLINICAL PRESENTATION -ROUTINE X-RAY -MEAN AGE 60 YEARS -COUGH, DYSPNEA, PAIN -PLEURAL EFFUSION

• PROGNOSIS -CELL TYPE -LOCALIZED OR DIFFUSE -STAGING MANDATORY

Case 6

I

Penrose Can<:er Conference 1997

Case#6 • Follow-up:

-At the time of diagnosis he was treated with radiation, which he tolerated well

- One year after presentation, the patient complained of dysphagia, dysphonia, generalized weakness and a right facial droop.

- hemoptysis and/or hematemesis

- physical exam revealed an oral mass

-increasingly lethargic

-expired after several days in the hospital; autopsy permission was not granted


Case#6

• DECISION POINTS -Obviously malignant: carcinoma, lymphoma,

melanoma?

-?Smoker

- If lymphoma--what is the subtype? • Epithelial invasion is present

• Some large pleomorphic cells present (? CD30 positive)

- Review transbronchial biopsy--could easily call large cell carcinoma

I

Penrose C3ncer Conference 1997

Case#6


Large cell Lymphoma

Dr. Colby's Diagnosis: Diagnosis: Large cell B cell lymphoma with

endobronchial involvement (and probably also hilar nodes).


Case#6

• LARGE CELL LYMPHOMAS IN THE LUNG - Large cell lymphoma, NOS (T, B, null) - Angiocentric lymphoma (lymphomatoid

granulomato.sis) - Intravascular lymphomatosis (angiotrophic lymphoma) - Anaplastic large cell lymphoma (CD.30 positive) - Post-transplant lymphoprolifer-ative disorders - "Opportunistic" lymphomas in the immunosuppressed

setting · - Transform alien of a low grade lymphoma/CLL - Hodgkin's disease - Myelogenous leukemia

Penrose Cancer Confefence 1997

Expert consultants Case #6

Askin Lymphoma vs carcinoma Chan Lymphoma Dail Sarcoma vs carcinoma Hammar Undifferentiated neoplams: lymphoma;

carcinoma; melanoma; angiosarcoma Koss- - ·-·carciilam·a-vs lymphom-aoiiTieianoma

Rosai Large cell/anaplastic lymphoma, need IHC Travis Poorly differentiated neoplasm; lymphoma

vs melanoma Weidner Lymphoma or carcinoma, need IHC ····----····---.. -·····'· "··-·····-·-·~·-·-·---·-·---·---.. ····-----· .. ·- -Yousem Large cell lymphoma

Penrose Cancer Conference ·1997

Participant diagnosis Case#6

Lymphoma, including Ki-1 lymphoma 8

Large cell undifferentiated carcinoma 4

Poorly differentiated carcinoma vs . Lymphoma 3 __ ,,.,..... .. ,...... .... ... ....... _._,........., ............... -·-··· Neuroendocrine carcinoma 2

Melanoma 2 -----·-·······-·--·······-··-- ·----·--····----·----- --- - --::--Atypical carcinoid 2

Undifferentiated malignant tumor 1

3




• CARCINOID TUMOR

• POORLY DIFFERENTIATED LUNG CANCER

• NEUROENDOCRINE CARCINOMA

Case 7

PARANEOPLASTIC SYNDROME

• CUSHING SYNDROME - ECTOPIC ACTH PRODUCTION

-WEIGHT LOSS

- DIABETES MELLITUS

-ELEVATED PLASMA ACTH

-24 HOUR URINE-FREE CORTISOL

• TREATMENT -RESECTION/CHEMOTHERAPY

Case 7


Case #7

• Follow-up: -discharged two months after surgery. -four months after surgery he was admitted for

two weeks for repair of the fistula and placement of a serratus anterior muscle flap over the closure site.

-visual inspection of the mediastinum was suspicious for recurrence

- patient refused a biopsy

Penro5e Cancer Conference 1997

Case#7

• Follow-up {cont): -during hospitalization he complained of left

scapular and left iliac chest pain. -four and 1/2 months after surgery a soft tissue

mass from the left scapula region was positive for metastatic disease.

-Radiotherapy was instituted. -expired 8 months after initial surgery.


• ISSUES Case #7

- Location--medial lower lobe extending to pleura

- ? Near mediastinum (? mediastinal tumor) - Benign versus malignant - Lung versus thymic versus mesothelial versus

other? - Distinctive features

• Uniformity of cells • Prominent cel l borders • Clear cell zones

- Management may be more clearcut than specific diagnosis


Case #7

Submitting Pathologist's Diagnosis: Large cell undifferentiated carcinoma with clear

cell features.

Dr. Colby's Diagnosis: Carcinoma, ? lung origin.

Ponrose Cancer Conforonce 1907


Askin Carcinoma Chan Carcinoma, probably squamous Dail Large cell neuroendocrine carcinoma vs

malignant carcinoid Hammar La rge cell neuroendocrine carcinoma

Koss Carcinoma with clear cell fea tures Rosai·-··· lung carcinoma exclude thymic -or ____ _

metastatic tumor Travis Squamous cell carcinoma 'wei'Cine.r Adenocarcinoma·v·s u·ndiffereni'iated-large·-

cell carcinom a Yousem Carcinoma with clear cell features, exclude

neuroendocrine carcinoma

Ptnrose Cancer Conference 1997

Case #7 Participant diagnoses

Large cell neuroendocrine carcinoma 7

Large cell undifferentiated carcinoma with clear cells 6 . ·····

Poorly differnetiated squamous cell carcinoma 1 .... _._... ,,__,................ . ___ . ., ........ --- ---·-·-- ··-- _ ........ . Neuroendocrine carcinoma rule out seminoma 1 .................................................................. ,,,_ ................. - ................................................................... . Malignant epithelial neoplasm - germ cell origin 1

Lung cancer High grade mucoepidermoid carcinoma

Atypical carcinoid ..

Poorly differentiated adenocarcinoma

1

1

1

1 ........................................ , .. .,. ...................................................... , ................................................................. .

J



• PRIMARY LEIOMYOMA

• HEMANGIOPERICYTOMA

• PULMONARY FIBROLEIOMYOMA

• BENIGN METASTASIZING LEIOMYOMA

Case 8

BENIGN METASTASIZING LEIOMYOMA

• PRIOR UTERINE SURGERY

• SINGLE/MULTIPLE

• WELL DEMARCATED

• DYSPNEA, COUGH, PAIN

• RESECT WHEN POSSIBLE

Case 8


HEMANGIOPERIC¥TOMA

• SOLITARY

• WELL DEMARCATED

• RARE IN LUNG

• COUGH, HEMOPTYSIS

• LOCALLY INVASIVE

Case 8

Penroi-t Cancer Conference 1997

Case#B

• Follow-up (provided by Dr. Bruce Brian, St. Joseph's Hospital, Denver, CO): - Patient was seen in August, 1997 and is doing

well -There is no evidence of recurrence or

metastatic disease -What is the clinical potential for this unusual

lesion?

Penrose cancer Conference 1997

Case #8

• DECISION POINTS - Epithelial versus mesenchymal

-Localized fibrous tumor versus hemangiopericytoma versus other

- Primary versus metastatic (remember stromal

sarcoma) - Benign versus malignant ?

Penrose Cancer Conte~tnce 1997

Case #8

• SARCOMAS IN THE LUNG - Practically any sarcoma encountered in the

soft tissue may be encountered in the lung. They are all rare.

- Sarcomatoid carcinomas may be mistaken as sarcomas.

Pervose Cancer Confertnc.e 1997

• HEMANGIOPERICYTOMATOUS TUMORS IN THE LUNG - Hemangiopericytoma, 1 o and 2•

- Localized fibrous tumor

-Other sarcomas, 1 o and 2•

-Spindled carcinoid tumors

-Miscellaneous (e.g., glomus tumor)

Case #8

Ptnrose Cancer Conference 1997

Case#S

Submitting Pathologist's Diagnosis: Hemangiopericytoma.

Dr. Colby's s Diagnosis: Hemangiopericytoma

Penrose C~r Conference 1997

Expert consultants Case #8

Askin Matastatic uterine stromal tumor - - --

Chan Metastatic endometrial stromal sarcoma

Dail Malastatic endometrial stomal sarcoma

Hammar Hemagiopericytoma; spindle cell sarcoma Koss Sarcoma, endometrial stromal sarcoma Rosai It looks like a vascular tumor of some sort.

Travis Endometrial stromal sarcoma vs sclerosing hemangioma

Weidner Hemangiopericytoma, RIO uterine ..... _ .... ___ ..J!I_E}.~~D..9 .. ~Y!!l .. ~!.tl:' mor .......... ·-- ·-·-··-- ···- - .......... _ Yousem Hemangioperlcytoma RIO stromal sarcoma

J

Penrose Cancer Conferenc.o 1997


Hemangiopericytoma 12 - ·---·-- - - ------·---Metastatic leiomyoma 2 Metastatic endometrial stromal sarcoma 2 --·----···-...... -.. -·-·-· ·--·----· Epithelial hemangioendothelioma 2 Thymoma 1 Synovial sarcoma 1

Spindle cell carcinoid tumor 1 Leiomyosarcoma 1 Inflammatory myofibroblastic tumor 1




• LIPOMA

• Ll POSARCOMA

• THYMOLIPOMA

Case 9

THYMOLIPOMA

• LARGE

• FAT AND THYMIC TISSUE

• MYASTHENIA RARE

• RESECT

• RECURRENCE RARE

Case 9

Pe('lrose <!ancer Conference 1997

Case#9

• Follow-up:

- Patient remains healthy -No evidence of reoccurrence

Penrose Cancer Conferenoe 1997

Case#9


Liposarcoma, well differentiated

Dr. Colby's Diagnosis: Low grade liposarcoma,? thymic origin.


Expert consultants

Askin

Chan

Dail

Hammar

Koss

Rosai

Liposarcoma

Liposarcoma

Sclerosing liposarcoma

Atypical lipoma



Travis Sclerosing liposarcoma

Case#9

....... ........................................................... , .................................................................................... . Weidner Well differentiated, liposarcoma

Yousem Liposarcoma

Pervo.se Cancer Conference 1997

Case #9 Participant diagnoses

Uposarcoma, well differentiated 11

Atypical lipoma 3

Thymolipoma 3

Spindle cell lipoma 2

Upoma 2




• ESOPHAGEAL DUPLICATION CYST

• DERMOID • TERATOMA

Case 10

TERATOMA

• CYSTIC AND BENIGN • THREE GERM CELL LAYERS • COMMONLY ANTERIOR • SYMPTOMS OF COMPRESSION

-PLEURA, PULMONARY ARTERY -BRONCHUS, VENA CAVA

Case 10

I

Penrose Cancer Confer!!' nee 1997

Case #10


Lipoma

Dr. Colby's Diagnosis: Mediastinal lipomatosis

(? pneumonia in LLL) Age 12 (slide review): Diffuse cartilagenous hamartoma with some features of placental transmogrification; ? fatty component


Case #10

• Fatty lesions of the mediastinum

• Previous material in case 9

I


Case #10

• FATTY LESIONS IN THE MEDIASTINUM - (+/-thymic origin)

- Lipomatosis -Lipoma

- Liposarcoma

- Thymolipoma (thymofibrolipoma)

- Others: lipoblastoma, lipoblastomatosis, hibernoma, angiolipoma

-? Fatty metaplasia of connective tissue

Penros.e Cancer Conference 1997

• MEDIASTINAL LIPOMATOSIS - Obesity

-Cushing's - Steroid therapy

- ? Replacement metaplasia

Case #10


• LIPOSARCOMA OF THE ANTERIOR MEDIASTINUM AND THYMUS*

Case #10

- 28 cases of anterior mediastinal liposarcoma

- Mean age: 43 years (14-72)

- 25% thought to arise in the thymus

- Majority low grade/well differentiated/sclerosing/myxoid

- Follow-up in 23 cases: 11 alive NED; 4 alive WD; 7 DOD; 1 postop death

- "Fatty tumors of the mediastinum should be regarded as most likely malignant..."

- ·Am J Surg Pathol 19:782, 1995 (Kiimstra et al)

Penrose Cancer Conference 1 gg7


Askin Mediastinallipomatosis ~~--~---------------Chan Lipoma/lipomatosis ----~------------------Dail Lipoma of esophagus ~~~~~~--~~~--

Hammar Hemangiomatosis - lymphangiomatosis Koss lipomatosis

--~----~--------------Rosai lipoma or lipomatosis ----~~----------------Travis Lipoma vs well differentiated liposarcoma

--- .... ---- - -·- ··-··----· ........ ··-··--·- - -·-··-·· - ......... ···--- ··"··-··· Weidner lipoma

------------------------Yousem Lipoma ------------------------

j

Penr(ISE! Cancer Conference 1"997

Case #10 Participant diagnosis

Teratoma 9

Lipoma/Lipomatosis 4 - ·····--·-··" ' " " '"""-"""""""'-"""-"""'""""'""""'"'""'--""'"-'"""' __ _ Fibrolipoma 2

Well differentiated liposarcoma 1 Pseudomediastinum 1

44_4_····-·-·-·-·-·-·-·-·-·-·-·--·--·-·-·-·-·-·-·-·--·-- ----·-·---···---···---·-·---· Hamartoma 1 Esophageal duplication 1

Angiomyolipoma 1




• METASTATIC ADENOCARCINOMA • ADENOCARCINOMA OF LUNG • BRONCHOALVEOLAR

CARCINOMA

Case 11

BRONCHOAL VEOLAR CARCINOMA

• SOLITARY OR DIFFUSE • MULTICENTRIC • SOLITARY GOOD PROGNOSIS • DIFFUSE USUALLY FATAL • SYMPTOMS

-WATERY SPUTUM -COUGH -TREATED FOR PNEUMONIA

• BRUSH AND BIOPSY Case 11


Case #1 1

• Follow-up: -As of September 10, 1997, patient is still living

- He is having a difficult time accepting that he has a terminal illness.

- He has not received any chemotherapy or radiation after his surgery in February 1997.

- He is at home under home hospice care, requires 02 and has episodes of hemoptysis.


Case #11


Mesothelioma

Dr. Colby's Diagnosis: Adenocarcinoma with intra-alveolar and

lymphang itic spread and pleural involvement. Most consistent with lung origin.

Penrose Cancer Conferer'lce 1997

Case#11

• ISSUES -Adenocarcinoma with spread versus

mesothelioma with lung invasion ? - Pseudomesotheliomatous carcinoma ? - Is this a bronchioloalveolar carcinoma (BAC) ?

-Exclusion of carcinoma from extrapulmonary source

- Growth pattern (BAD-like) and cytology

Penrose Canoer C<mferenee 1997

Case #11

• BRONCHIOLOALVEOLAR CARCINOMA

• Definition -Growth of tumor cells along pre-existing ll.lng

architecture without interstitial or lymphatic invasion

-Many primary carcinomas of the lung show foci (sometimes large) of bronchioloalveolar growth pattern; metastatic carcinomas may also show focal growth along alveolar walls.

Expert consultants P•nrDit CancerConf•r•nce1997

. 1 . RJO h Case#11 Askin Papillary caranoma; ung pnmary - t yro1d ca Chan Adenocarcinoma with intranudear indusions,

____ consist\lntv.ith !}'p_eJ! PQilJ:!mQcy!e differenti.§ltio_ll_ Dail Adenocarcinoma, with extensive lymphangietic

spread raising the possiblity of this being a secondary tumor

Hammar Adenocarcinoma of type II pneumocyte origin

··-·-·-· .. ·-·-~.!J~!29.1c>.~L~9 .. P..~!!.\ll!l .... _ ___ ,_·--·--·----Koss Papillary adenocarcinoma Rosai Adenocarcinoma v.ith broncholoalveolar/Ciara cell

features Travis Adenocarcinoma, mixed type v.ith components of

·-·---·--~-nar ,_§did l!nd_l?f.QI.lchloloal~eola~n::::o.::.m:::a~Weidner Bronchioloalveolar-cell adenocarcinoma (Type II) Yousem Papillary adenocarcinoma

Penrose Cancer Con'ferenct 1997

Case #11 Participant diagnosis

Bronchoalveolar carcinoma 9 Adenocarcinoma 4

Bronchioalveolar carcinoma, non mucinous 3

Papillary adenocarcinoma 2

Lung cancer 1

Mesothelioma - rule out adenocarcinoma 1

Peripheral adenocarcinoma 1

3




• MESOTHELIOMA • THYMIC CARCINOMA

Case 12

THYMIC CARCINOMA

• LOW GRADE AND HIGH GRADE • CLINICAL PRESENTATION

-AVG. AGE 45 YRS -PAIN, DYSPNEA, VENA CAVA -ASYMPTOMATIC (30%)

Case 12

I


THYMIC CARCINOMA

• TREATMENT -RESECT -CHEMOTHERAPY/RADIATION

• SURVIVAL -BASED ON HISTOLOGIC TYPE - BASED ON STAGE -5 YEAR (33%)

Case 12

MESOTHELIOMA

• CLINICAL PRESENTATION -PAIN, DYSPNEA -ASBESTOS EXPOSURE -PLEURAL EFFUSION -STARTS AT BASE

• EPITHELIOD/SARCOMATOUS

Case 12



MESOTHELIOMA

• TREATMENT -EXTRAPLEURALPNEUMONECTOMY - PLEURECTOMY -TALC SCLEROSIS -OBSERVE -CHEMOTHERAPY/RADIATION

• PROGNOSIS -FATAL

Case 12

3


Case #12

• Follow-up:

-Patient expired, tissue was from an autopsy.


Case #12

Submitting Pathologist's Diagnosis: Pseudomesotheliomatous carcinoma


Large cell carcinoma.

I


• DISCUSSION - Obviously malignant -- problem is

classification

- Carcinoma versus mesothelioma ?

Case #12

- Is this a pseudomesotheliomatous carcinoma ?

- Separation of carcinoma from mesothelioma

Penrose Ca.neer Conrerenct 1997

• PSEUDOMESOTHELIOMATOUS CARCINOMA

• Definition

Case #12

- Dail & Hammar (2nd edition): " ... peripheral lung neoplasms that for reasons unknown invaded into the pleura and grew like a mesothelioma."

P•nro'"' Cancer Conference 1997

Case #12

• PSEUDOMESOTHELIOMATOUS CARCINOMA

- Definition

- AFIP Fascicle (Battifora and McCaughey): "The primary tumor may be overshadowed by the pleural-based growth in some cases, and the term pseudomesotheliomatous adenocarcinoma has been proposed for such cases. Peripheral lung cancers resembling bronchiolar carcinoma have a particular propensity to imitate mesothelioma, but oat cell carcinoma and other histologic types may do so as well, albeit rarely."

Penrose Cancer Confeftnce 1997

• MESOTHELIOMA VERSUS METASTATIC CARCINOMA - Gross/radiologic findings

Case #12

-(But localized mesotheliomas are recognized)

- Routine histology -History of prior tumor (compare slides)

- (But XRT may cause mesothelioma)

- Histochemistry (mucins)

-Immunohistochemistry

-Mesothelioma-specific antibodies available

- Electron microscopy

J

Penro" Cancer Conference 1997

Case #12

• ORIGINAL IMMUNOHISTOCHEMISTRY

• (Dr. Shah) - CEA 50% of cells positive - BER-EP4 70% of cells positive - B72.3 Negative

Expert consultants Penrose Cancer Conrtrence 1997

Askin Pseudomesotheliomatosus lung carcinoma Case #12 Chan Malignant neoplasm. favor malignant mesothelioma.

confirm with EM & IHC Daif Malignant neoplasm-wiih some rhabdoicffea.lures:·· ······-··

questionably with striations. May be a rhabdoid carcinoma_L.exctude with IHC

Ha._m_ m_a_r -7'M:"a7:1ig!!!.nant pleural neoplasm: mesothelioma; epitheloid hemangioendothelioma; melanoma; renal cell carcinoma

Koss ______ Favor· mauij'ii'ii'iii"ili'esotheiio'iiia'"-... - ... -...... _ ... --·-.. ·--Rosai Anaplastic malignant tumor. I suspect it is a

___ £8rcinom!._!!_\!! one w.£.1!1d n eed tH~--- . Travis Poorly differentiated malignant neoplasm with rh~a-:-b-,.do"'7id""

phenoty e Weidner Malignant mesothelioma, carcinoma

(pseudomesotheliomatous type), sarcoma, and even .. _ ..................... :!II!l.ll9.~.a.n.Lr.M.!?.!!.<?!!:IJ.\!!Tl9.f f!1uS!Jl!! consld~f-~!L ............. . Yousem Large cell carcinoma with pleural involvement


Participant diagnoses Case #12

Mesothelioma. 12 ---------------------------------Large cell undifferentiated carcinoma 6

Metastatic melonoma 3

Undifferentiated malignant tumor, ? rhabdoid 2 tumor Mesothelioma vs carcinoma 1

Lymphoma 1 ··-·-- ------ ---- ----

Granulocytic sarcoma 1

5




• NEUROFIBROMA • BENIGN MESOTHELIOMA • FIBROMA OF LUNG • FIBROUS TUMOR OF PLEURA

Case 13

FIBROUS TUMOR PLEURA

• CLINICAL PRESENTATION -SLOW GROWING -NOT RELATED ASBESTOS -ASYMPTOMATIC MASS - BECOME LARGE -CLUBBING -HYPOGLYCEMIA

Case 13

I

Penrose .Cancer Conference 199j

L. Penfield Faber, MC

FIBROUS TUMOR PLEURA

• DIAGNOSIS -NEEDLE BIOPSY POOR -RESECTION

• SURGERY -WEDGE OF LUNG ADEQUATE -LARGE TUMOR IS VASCULAR -RECURRENCE RARE

Case 13

Ptnrose Cancer Conference 1 !il97

Case #13

• Follow-up: - Patient is doing well with no recurrance

Penrose C1noer Conference 1997

Case #13


Solitary pleural nodule

Dr. Colby's Diagnosis: Localized fibrous tumor (LFT).

I

Penrose Canc:er Conrtrenoe t997

Case #13

• LOCALIZED FIBROUS TUMOR (LFT) -The rising star of LFT

-A soft tissue tumor occurring in a variety of sites

- Intrathoracic • Pleural • Intrapulmonary

• Mediastinal

- Criteria of malignancy

- Differential diagnosis


• CRITERIA OF MALIGNANCY IN INTRAPULMONARY LFT* -Two or more of:

• Necrosis

• Mitotic activity (>4/1 0 hpf)

• Vascular invasion

• High cellularity - *Wilson et al

Case #13

Penrose C.anoer Conference 1997

• APPROACH -Spindle cell tumor

-Histologically benign

-Variable cellularity - "patternless pattern"

- Destructive collagen

Case #13

Penrose Cancer Conftrtnce 1997

Expert consultants

Askin

Chan

Fibrous pleural tumor Solitary fibrous tumor

Dail Fibrous tumor of pleura

Hammar Fibrous tumor of pleura

Case #13

----

Koss Localized fibrous tumor of pleura Rosai Solitary fibrous tumor of pleura Travis Fibrous tumor of pleura

·-----···---Weidner Solitary fibrous tumor -·- -- --- ----- ---- ---- - ··----Yousem Localized fibrous tumor

3

Participant diagnoses

Solitary fibrous tumor

Penrose Cancer Conierence 1997

Case #13

9 Localized fibrous tumor of the pleura 6

Leiomyoma 2

Pleural fibroma 2

Fibrosarcoma 1 Fibrous mesothelioma 1 Mesothelioma 1 Sarcomatoid mesothelioma 1



DIFFUSE DISEASE WHICH REQUIRES OPEN BIOPSY

• DIFFUSE ALVEOLAR DAMAGE (DAD) • USUAL INTERSTITIAL PNEUMONITIS (AlP) • BRONCHIOLITIS OBLITERANS • BRONCHIOLITIS WITH ORGANIZING

PNEUMONIA

DIFFUSE DISEASE WHICH REQUIRES OPEN BIOPSY

• RHEUMATOID, SCLERODERMA, LUPUS • INFECTIOUS GRANULOMA • THROMBOEMBOLI • ARTERIAL HYPERTENSION • VENO-OCCLUSIVE DISEASE

I

Penrose Cancer Conference 19!

L. Penfield Faber, ,

DIFFUSE DISEASE WHICH MAY REQUIRE LARGE WEDGE OR SEGMENTECTOMY

• PNEUMONONIOSIS • BRONCHIECTASIS • BRONCHOCENTRIC

GRANULOMATOSIS • NECROTIZING SARCOIDAL

GRANULOMATOSIS • WEGENER'S DISEASE

1997 PENROSE CANCER CONFERENCE TUMORS OF THE CHEST

REFERENCES FOR DR. COLBY

Case I: Lymphangioleiomyomatosis

I. Bonetti F, Chiodera P. Lymphangioleiomyomatosis and tuberous sclerosis: Where is the border? Eur Respir J 9:399-401, 1996.

2. Bonetti F, Chiodera PL, Pea M, Martignoni G, Bosi F, zamboni G, Mariuzzi GM. Transbronchial biopsy in lymphangiomyomatosis of the lung. Am J Surg Pathol 17:1092-1 102, 1993.

3. Castro M, Shepherd CW, Gomez MR, Lie JT, Ryu JH. Pulmonary tuberous sclerosis. Chest 107:189-195, 1995.

4. Corrin B, Liebow AA, Friedman PJ. Pulmonary lymphangioleiomyomatosis. A review. Am J Patho1 79:347-382.

5. Crausman RS, Jennings CA, Monenson RL, Ackerson LM, Irvin CG, King TE Jr. Lymphangioleiomyomatosis: The pathophysiology of diminished exercise capacity. Am J Respir Crit Care Med 153:1368-1376, 1996.

6. Ka1assian KG, Doyle R, Kao P, Ruoss S, Raffin TA. Lymphangio1eiomyomatosis: New insights. Am J Respir Crir Care Med 155:1183-1186, 1997.

7. Kitaichi M, Nishimura K, Itoh H, Izumi T. Pulmonary lymphangioleiomyomatosis: A repon of 46 patients including a clinicopathologic study of prognostic factors. Am J Respir Crit Care Med 151:527-533, 1995.

8. O'Brien JD, Lium JH, Parosa JF, DeYoung BR, Wick MR, T rulock EP. Lymphangiomyomatosis recurrence in the allograft after single-lung transplantation. Am J Respir Crit Care Med 151:2033-2036, 1995.

9. Taylor JR, Ryu J, Colby TV, Raffin TA. Lymphangioleiomyomatosis. Clinical course in 32 patients. NEJM 323:1254-1260, 1990.

Case 2: Metastatic Angiosarcoma

I. Palvio DHB, Paulsen SM, Henneberg EW. Primary angiosarcoma of the lung presenting as intractable hemoptysis. Thorac Cardiovasc Surgeon 35:105-107, 1987.

2. Patel AM, Ryu JH. Angiosarcoma in the lung. Chest 103:1531-1535, 1993.

T. Y. Colby, M.D. -2 -

3. Segal SL, Lenchner GS, Cichelli A Y, Promisloff RA, Hofman Wl, Baiocchi GA. Angiosarcoma presenting as diffuse alveolar hemorrhage. Chest 94:214-218, 1988.

4. Sheppard MN, Hansell DM, DuBois RM, Nicholson AG. Primary epithelioid angiosarcoma of the lung presenting as pulmonary hemorrhage. Hum Pathol 27:383-385, 1997.

5. Spragg RG, Wolf PL, Haghighi P, Abraham JL, Astarita RW. Angiosarcoma of the lung with fatal pulmonary hemorrhage. Am J Med 74:1072-1076, 1983.

6. Yousem SA. Angiosarcoma presenting in the lung. Arch Pathol Lab Med 110:112-115' 1986.

Case 3: Pulmonary Langerhans Cell Histioq1osis

I. Colby TV, Lombard C. Histiocytosis X in the lung. Hum Pathol 14:847-856, 1983.

2. Delobbe A, Durieu J, Duhame A, Wallaert B. Determinants of survival in pulmonary Langerhans' cell granulomatosis (histiocytosis X). Eur Respir J 9:2002-2006, 1995.

3. Friedman PJ, Liebow AA, Sokoloff J. Eosinophilic granuloma of the lung: Clinical aspects of primary histiocytosis in the adult. Medicine 60:385-396, 1981.

4. Housini I, Tomashefski JF, Cohen A, Crass J, Kleineman J. Transbronchial biopsy in patients with pulmonary eosinophilic granuloma. Comparison with findings on open lung biopsy. Arch Pathol Lab Med 118:523-530, 1994.

5. Soler P , Kambouchner M, Valeyre D, Hance AJ. Pulmonary Langerhans' cell granulomatosis (histiocytosis X). Annu Rev Med 43: 105-1 15, 1992.

6. Soler P, Moreau A, Basser F, Hance AJ. Cigarette smoking-induced changes in the number and differentiated state of pulmonary dendritic cells/Langerhans cells. Am Rev Respir Dis 139:1112-1117, 1989.

7. Travis WD, et al. Pulmonary Langerhans cell granulomatosis (Histiocytosis X): A clinicopathologic study of 48 cases. Am J Surg Pathol17:97l-986, 1993.

8. Willman CL, Busque L, Griffith BB, Favara BE, McClain KL, Duncan MH, Gilliland DG. Langerhans' cell histiocytosis (histiocytosis X): A clonal proliferative disease. NEJM 331:154-160, 1994.

T. V. Colby, M.D. - 3-

Case 4: Low Grade T Cell Lymphoma

I. Colby TV, Koss MN, Travis WD. Tumors of the Lower Respiratory Tract. In: Atlas of Tumor Pathology. Third Series, Fascicle 13, AFIP, Washington DC, 1995.

2. Ono K, Shimamoto Y, Katsuzaki M, Sano M, Yamaguchi T , Kato 0, Yamada H, Yamaguchi M. Diffuse panbronchiolitis as a pulmonary complication in patients with adult T cell leukemia. Am J Hematol 30:86-90, 1989.

3. Yoshioka R, Yamaguchi K, Yoshinaga T, Takatsuki K. Pulmonary complications in patients with adult T cell leukemia. Cancer 55:2491-2494, 1985 .

Case 5: Lymphomatoid Granulomatosis/ Angiocentric Lymphoma

I. Guinee D , Jaffe E, Kingma D, et al. Pulmonary lymphomatoid granulomatosis. Evidence of r a proliferation of Epstein-Barr virus infected B lymphocytes with a prominent T cell component and vasculi tis. Am J Surg Pathol 18:753-764, 1994.

2. Liebow A, Carrington C, Friedman P. Lymphomatoid granulomatosis. Hum Patho1 3:457-558, 1972.

3. Lipford E Jr, Margolick J, Longo D, et al. Angiocentric immunoprolife rative lesions: A clinicopathologic spectrum of post-thymic T cell proliferations. Blood 72:1674-1681, 1988.

4. Medeiros L, Jaffe E, Chen Y-Y, Weiss L. Localization of Epstein-Barr viral genomes in angiocentric immunoproliferative lesions. Am J Surg Pathol 16:439-447, 1992.

5. Myers JL, Kurtin PJ, Katzenstein A-LA, Tazelaar HD, Colby TV, Strickler JG, Lloyd RV, Isaacson PG. Lymphomatoid granulomatosis. Evidence of immunophenotypic diversity and relationship to Epstein-Barr virus infection. Am J Surg Pathol 19:1300-1312, 1995.

Case 6: Large Cell Lymphoma

I. Colby TV, Koss MN, Travis WD. Tumors of the Lower Respiratory T ract. In: Atlas of Tumor Pathology. Third Series, Fascicle 13, AFIP, Washington DC, 1995.

Case 7: Poorly Differentiated Carcinoma

I. Colby TV, Koss MN, Travjs WD. Tumors of the Lower Respiratory Tract. In: Atlas of Tumor Pathology. Third Series, Fascicle 13, AFIP, Washington DC, 1995.


Case 8: Hemangiopericytoma

1. Meade JB, Whitwell F, Bickford BJ, Waddington JKB. Primary haemangiopericytoma oflung. Thorax 29:1-15., 1974.

2. Shin Myun Soo, Ho K-J. Primary hemangiopericytoma of lung: Radiography and pathology. AJR 133:1077-1083, 1979.

3. Yousem SA, Hochholzer L. Primary pulmonary hemangiopericytoma. Cancer 59:549-555, 1987.

Cases 9 and 10: Lipomas/Liposarcoma of the Mediastinum

I. Cicciarell i FE, Soule EH, McGood DC. Lipoma and liposarcoma of the mediastinum~ A report of 14 tumors including one lipoma of the thymus. J Thorac Cardiovasc Surg 47:411, 1964.

2. Dogan R, Ayrancioglu K, Aksu 0 . Primary mediastinal liposarcoma. A report of a case and review of the literature. Eur J Catdiothorac Surg 3:367-3770, 1989.

3. Klimstra DS, Moran CA, Perino G, Koss MN, Rosai _J. Liposarcoma of the anterior mediastinum and thymus. A clinicopathologic study of 28 cases.. Am J Surg Pathol 19:782-791 , 1995.

4. Moran CA, Zeren H, Koss MN. Thymofibrolipoma. A histologic variant of thymolipoma. Arch Pathol Lab Med 118:281-282, 1994.

5. Pachter MR, Lattes R. Mesenchymal tumors of the mediastinum. I. Tumors of fibrous tissue, adipose tissue, smooth muscle, and striated muscle. Canc.er I 6:74-94, 1963.

6. PanCH, Chiang CY, Chen SS. Thymolipoma in patients with myasthenia gravis: Report of two cases and review. Acta Neural Scand 78:16-21 , 1988.

7. Rosai J, Levine GD. Atlas of tumor pathology, 2nd series, Fascicle 13. Tumors of the. thymus. Washington DC. AFIP, 162-166, 1976.

Cases 11 and IZ: Pseudomesotheliomafous Carcinoma/Mesothelioma

I. Bedrossian CWM, Bonsiq S, Moran. C. Differential diagnosis between mesothelioma · and adenocarcinoma: A multi modal approach based on ultrastructure and immunocytochemistry. Sem Diag Pathol 9: 124-140, I 992.


2. Doglioni C, Dei Tos AP, Laurino L, Iuzzolino P, Chiarelli C, Celio MR, Viale G. Am J Surg Pathol 20:1037-1046, 1996.

3. Koss M, Travis W, Moran C, Hochholzer L. Pseudomesotheliomatous adenocarcinoma: A reappraisal. Sem Diag Pathol 9:117-123, 1992.

4. Leong A S-Y, Vernon-Roberts E. The immunohistochemistry of malignant mesothelioma. Pathol Annu 29(part 2): 157-181, 1994.

5. MacDougall DB, Wang SE, Zidar BL. Mucin-positive epithelial mesothelioma. Arch Pathol Lab Med I 16:874-880, 1992.

6. McCaughey WTE, Colby TV, Battifora H, Churg A, Corson JM, Greenberg SD, Grimes MM, Hammar S, Roggli VL, Unni KK. Diagnosis of diffuse malignant mesothelioma: Experience of a US/Canadian mesothelioma panel. Mod Pathol 4:342, 1991.

7. Miettinen M, Kovatich AJ. HBME-1 a monoclonal antibody useful in the differential diagnosis of mesothelioma, adenocarcinoma, and sofi tissue and bone tumors. Appl Immunohistochem 3:115-122, 1995.

8. Ordonez NG, Mackay B. The roles of immunohistochemistry and electron microscopy in distinguishing epithelial mesothelioma of the pleura from adenocarcinoma. Adv Anat Pathol 3:273-293, 1996.

9. Sheibani K, Esteban JM, Bailey A, Battifora H, Weiss LM. Immunopathologic and molecular studies as an aid to the diagnosis of malignant mesothelioma. Hum Pathol 23:107-11 6, 1992.

Case 13: Localized Fibrous Tumor

1. Briselli M, Mark EJ, Dickersin GR. Soli tary fibrous tumors of the pleura: Eight new cases and review of 360 cases in the literature. Cancer 47:2678-2689, 1981.

2. el-Nagger AK, Ro JY, Ayala AG, Ward R, Ordonez NG. Localized fibrous tumor of the serosal cavities. Immunohistochemical, electron microscopic, and flow cytometric DNA study. Am J Clio Pathol 92:561-565, 1989.

3. England DM, Hochholzer L, McCarthy MJ: Localized benign and malignant fibrous tumors of the pleura. A clinicopathologic review of 223 cases (published erratum appears in Am J Surg Pathol August 1991; 15:818). Am J Surg Patholl3:640-658, 1989.


4. Hanau CA, Miettinen M. Solitary fibrous tumor: Histological and immunohistochemical spectrum of benign and malignant variants presenting at different sites. Hum Pathol 26:44.0-449, 1995.

5. LeeKS, Jm JG, Choe KO, Kim CJ, Lee BH. CT findings in benign fibrous mesothelioma of the pleura: Pathologic correlation in nine patients. AJR 158:983-986, 1992.

6. Said JW, Nash G, Banks-Schlegel S, Sljssoon AF, Shintaku IP. Localized fibrous mesothelioma: An immunohistochemical and electron microscopic study. Hum Pathol 15:440-443, 1984.

7. Weidner N. 'solitary fibrous tumor of the mediastinum. Ultrastruct Pathol 15:489-492, 1991.

8. Wilson RW, Fishback N, Colby TV, de Christensen MR, Fleming MY, Koss MN, Travis WD: Intrapulmonary locali.zed fibrous tumor: A clinico·path'ologic analysis of 43 cases. Modem Pathol 8:155A, 1996.

9. Witkin GB; Rosai J. Solitary fibrous tumor of the mediastinum. A report of 14 cases. Am J Surg Pathol 13:547-557, 1989.

10. Yousem SA, Flynn SD. Intrapulmonary localized fibrous tumor. Intraparenchymal so-called localized fibrous mesothelioma. Am J Clin Pathol 89:365-369, 1988.

TVC:bp 8/29/97 q:'"is.l

Documents

History of the Penrose Cancer Conference · Jochelson, CA Forscher 1995 Prostate and Testicular Cancer Jl Epstein, RD McLeary, JA Smith, Jr., TM Ulbright, AT Stavros ... 25 East Jackson