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Primary tumors of bone Primary tumors of bone Mr Sreeram Penna www.eyst.org

Primary Tumors Of Bone

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Page 1: Primary Tumors Of Bone

Primary tumors of bonePrimary tumors of boneMr Sreeram Pennawww.eyst.org

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Malignant Tumors of BoneMalignant Tumors of BoneOsteosarcomaChondrosarcomaEwing SarcomaChordomaAdamantinomaMalignant Vascular tumorsMalignant fibrous histocytoma and

fibrosarcomaMultiple myeloma, Plasmacytoma

and LymphomaMetastatic bone tumors


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Benign/Aggressive tumorsBenign/Aggressive tumorsGiant cell tumorChondroblastomaChondromyxoid fibromaOsteoblastomaLangerhans cell histiocytosis


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Benign tumors Benign tumors Bone-forming Osteoid Osteoma Bone island

Cartilage Lesions Chondroma Osteochondroma

Fibrous lesions Non ossifying fibroma Cortical desmoid Benign fibrous histocytoma Fibrous displasia Osteofibrous dysplasia Desmoplastic fibroma


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Benign tumors contBenign tumors contCystic Lesions Unicameral bone cyst Aneurismal bone cyst Intraosseous ganglion cyst Epideromoid cyst

Fatty tumors Lipoma

Vascular tumors Hemangioma


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Non neoplastic conditionsNon neoplastic conditions Pagets disease Brown tumor of hyperparathyroidism Bone infarct Osteomyelitis Stress fracture Posttraumatic osteolysis


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Principles of mx - Principles of mx - InvestigationsInvestigations Plain radiographs of the bone CT scans MRI Bone scans Chest X-ray CT of chest


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GradingGrading Done histologically Biopsy need to be performed by team

prepared to do the resection.


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OsteosarcomaOsteosarcoma Tumor characterised by production of osteoid by

malignant cells. 2nd most commonest primary bone tumor, 20%

primary malignancies,1-3 per 1 million population. Age: Primary high grade osteosarcoma – 10 – 20

yrs , Parosteal osteosarcoma – 3rd and 4th decade, Secondary osteosarcoma in older population

M>F (except parosteal osteosarcoma) Genetic association: Rothmund-Thompson

syndrome, Li-Fraumani syndrome, hereditary form of retinoblastoma.

Location: distal femur, proximal tibia, and proximal humerus.


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OsteosarcomaOsteosarcoma Symptoms: Progressive pain, night pain or

painless mass. Mostly metaphyseal, 10% diaphyseal, 1%

epiphyseal Classification : - Primary Osteosarcoma: conventional low grade intramedullary parosteal periosteal high grade surface, telangectic small cell - Secondary Osteosarcoma


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Conventional Conventional OsteosarcomaOsteosarcoma High grade Common type of

Osteosarcoma Common radiographic

appearance is aggressive lesion producing osteoid matrix. Periosteal reaction may take the form of “codmans triangle” or “sunbrust” or “hair on end” appearence


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Periosteal OsteosarcomaPeriosteal Osteosarcoma Intemidate grade Arises from surface

of bone Commonly on femur

and tibia.


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Intramedullary Intramedullary OsteosarcomaOsteosarcomaRareLow grade


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Parosteal OsteosarcomaParosteal Osteosarcoma Low grade

malignancy Rare Arises on surface of

bone and invades medullary cavity only at later stages.

It has peculiar tendency to occur as a lobulated mass on the posterior aspect of femur


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High grade surface High grade surface OsteosarcomaOsteosarcoma High grade Least Common Radiographs show

invasive lesions with with ill defined borders


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Telangectic OsteosarcomaTelangectic Osteosarcoma Lytic lesion


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Small cell OsteosarcomaSmall cell Osteosarcoma Rare High grade Resemble Ewing

sarcoma or Lymphoma


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Secondary OsteosarcomaSecondary Osteosarcoma These occur at the site of another disease

process Older patients E.g. Pagets disease, previous radiation

treatment, fibrous dysplasia, bone infarcts, osteochondromas, chronic osteomyelitis, dedifferentiated chondrosarcomas, melorhestosis and osteogenesis imperfecta.


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InvestigationsInvestigations Plain radiographs CT scan Bone scan MRI Chest radiographs and CT for staging


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Osteosarcoma treatmentOsteosarcoma treatment Neoadjuvant chemotherapy Wide or radical surgery (resection or

amputation) Adjuvant chemotherapy


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ChondrosarcomaChondrosarcoma 9% of primary malignancies of bone Age: broad, primary chondrosarcoma peak

around 40 – 60yrs, secondary chondrosarcoma 25 – 45 yrs.

Any location but common around pelvis, proximal femur, proximal humerus.

Most common malignancy in hand. Clinically: increasing pain and palpable mass.

Pain in absence of pathological fracture is helpful to differentiate between enchondroma and low grade chondrosarcoma


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Secondary Secondary ChondrosarcomaChondrosarcoma Olliers disease (multiple enchondromatosis) Maffuci syndrome (multiple enchondromatosis

+ soft tissue haemangioma) Multiple hereditary exostoses Solitary osteochondroma Synovial chondromatosis Chondromyxoid fibroma Periosteal Chondroma Chondroblastoma Previous radiation treatment Firbours dysplasia


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Chondrosarcoma - xraysChondrosarcoma - xraysAppearence similar to

enchondroma, it is a lesion arising in medullary cavity with irregular matrix calcification. Pattern is described as “punctate,” “popcorn,” or “comma-shaped”. Compared with enchondroma, chondrosarcoma has more aggressive appearence with bone destruction and cortical erosions, periosteal reaction, and rarely soft-tissue mass.


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Mesenchymal Mesenchymal chondrosarcomachondrosarcoma


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Clear cell chondrosarcomaClear cell chondrosarcoma

Epiphyseal Gaint Cell Tumor Chondroblastoma Clear cell chondrosarcoma


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Dedifferentiated Dedifferentiated chondrosarcomachondrosarcoma

Radiographic features of dedifferentiated chondrosarcoma often show a more aggressive radiolucent area juxtaposed on a otherwise typical chondrosarcoma.


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Chondrosarcomas Chondrosarcomas TreatmentTreatment Low grade – Extended curettage with use of

intraoperative adjuvant treatment. High grade – Wide or radical resection or

amputation. Radiotherapy as palliative for inaccessible



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Ewing sarcomaEwing sarcoma 4th most common primary malignancy 9% of primary malignancy of bone Age: most occur in 5 – 25 yrs Commonly metaphyses of long bones(often

extension to diaphyses) and flat bones of shoulder and pelvic girdle.

Males > Females Clinically: Pain(insidious onset, mild and

intermittent initially), fever, erythema, swelling.

Investigation: elevated ESR, raised CRP


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Ewing sarcoma – xrays Ewing sarcoma – xrays

Destructive lesion in the diaphyses of longbone with and “onion skin” periosteal reaction.


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Ewing sarcoma - MRIEwing sarcoma - MRI To evaluate full

extent of lesion


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Ewing sarcoma - Ewing sarcoma - investigations investigations CXR CT chest Bone scan Bone marrow aspirate


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Ewing sarcoma – Ewing sarcoma – treatmenttreatment Radiosensitive Large central unresectable mass –

radiotherapy. Smaller more accessible lesions surgery. Neo adjuvant and adjuvant chemotherapy


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ChordomaChordoma Rare malignant neoplasm arises from notochord

remnants. Second most common in spine, most common in

sacrum. 50% of chordomas arise in sacrococcygeal region,

30% arise in base of the skull. Age sacrococcygeal 50 – 70 yrs spenooccipital 40 –

60 yrs Clinically: spenooccipital: headaches, symptoms of

cranial nerve compression, retropharyngeal abscess. Sacrococcygeal: lower back pain, sciatic pain, bowel and bladder problems. Palpable mass on PR. Spinal: presents with nerve root or cord compression.


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Chordoma radiologyChordoma radiologyDestructive lesions, virtually

arising from midline.


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AdamantinomaAdamantinoma Arises from aberrant

epithelial cells. Prediliction to tibia 2nd and 3rd decade Clinically pain and

palpable mass. Multiple sharply

demarcated lesions in tibial diaphyses

DD: Osteofibrous dysplasia. (diff by aggressive appearance)


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Malignant Vascular Malignant Vascular tumorstumorsHemangioendoth

elioma – low grade

Angiosarcoma – high grade


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Malignant fibrous Malignant fibrous histocytoma and histocytoma and fibrosarcomafibrosarcoma 3 – 5 % primary bone

malignancies All ages except 1st decade Both sexes affected equally Tendency to occur in distal

femur and proximal tibia. 25% considered secondary

from paget’s disease, irradiation, giant cell tumor and bone infarction.

Clinically presents with pain and pathological fracture(20%)

Radiologically aggressive appearance and are typically purely lytic with indistinct borders.


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Giant Cell TumorGiant Cell Tumor5% of bone neoplasmsAge: 20 to 40 yrsFemale>MaleLocation: distal femur> proximal tibia >

distal radiusBenign but pulmonary metastasis can occurClinically: progressive pain, pathological

fractures(10 – 30 %)Radiologically lesions are eccentrically

located in the epiphysis of long bones and usually abut subchondral bone. Lesions are purely lytic.

MRI useful for determining extent of disease.


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Giant cell tumor – x raysGiant cell tumor – x rays


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Giant cell tumor – MRI Giant cell tumor – MRI Dark in T1 weighted image and Bright in T2 weighted images


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Giant cell tumor – Giant cell tumor – treatment treatment Aggressive extended curettage and use of

adjuvants like argon beam coagulator. And fill the bone cavity with cement or bone graft

Inoperable lesions in pelvis use radiation or embolisation.

Follow up is very imp as lesions can reccur.


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ChondroblastomaChondroblastoma Age: 10 – 25 yrs M:F is 2:1 Commonly distal femur, proximal humerus, and

proximal tibia. Older patients flat bones Clinically: progressive pain Radiologically: well circumscribed lesion centered in

epiphyses of long bone, or an apophysis like greater trochanter or greater tuberosity. Often it has surrounding rim of reactive bone.

In children well circumscribed epiphyseal lesion that crosses open growth plate is diagnostic. In Adults DD is giant cell tumor and clear cell chondrosarcoma.

Treatment is extended curettage and adequate follow-up


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Chondroblastoma - adultsChondroblastoma - adults


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Chondroblastoma - kidsChondroblastoma - kids


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Chondromixoid FibromaChondromixoid Fibroma 10 – 30 yrs Commonly proximal tibia Clinically pain or swelling Xrays – well circumscribed lesion with rim of

sclerosis in metaphyses of long bone. Intra lesional calcium is absent.

Treatment – resection or extended curettage with bone grafting


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Chondromixoid FibromaChondromixoid Fibroma


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OsteoblastomaOsteoblastoma Bone forming neoplasm 10 – 30 yrs old M:F is 3:1 Any bone but commonly in spine Clinically: Pain worse at night relived by NSAID, in

spine scoliosis or root or cord compression. Xrays: Spine it shows bone forming neoplasm in

posterior elements(DD aneurismal bone cyst and Osteoid osteoma). Out side spine calcified central nidus with surrounding radiolucent halo and reactive sclerosis. (DD Osteoid osteoma)

Treatment extended curettage or resection. In spine may require instrumented fusion.


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Langerhans Cell Langerhans Cell HistiocytosisHistiocytosis Also known as Histocytosis X Isolated bony lesions called as eosinophilic granuloma 5 – 20 yrs Clinically progressive pain, fever, local signs of

inflammation Commonly affects vertebral bodies, flat bones and

diaphyses of long bones. X-rays – in spine vertebra plana. In flat bones lesions

are well circumscribed and “punched out” purely lytic lesions. In diaphyses of long bones aggressive permeative appearance with periosteal reactive bone formation.

Biopsy is required for diagnosis Treatment conservative (steroid therapy, radiation,



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Langerhans Cell Langerhans Cell HistiocytosisHistiocytosis


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Osteoid osteomaOsteoid osteoma 2nd and 3rd decades of life Any bone, but femur and tibia commonly cortical or cancellous Typically pain worse at night relieved by

aspirin or NSAID. Radiologically lesion consists of a small (< 1.5

cm) central nidus with surrounding bony sclerosis

Treatment – medical treatment, percutaneous radiofrequency ablation, or surgical removal


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Osteoid osteomaOsteoid osteoma


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Bone Island – Enostoses Bone Island – Enostoses Benign lesions of cancellous bone Any bone Asymptomatic Osteopoikilosis is multiple bone islands

through out skeleton X rays: typically small, round or oval areas of

homogeneous increased density with in cancellous bone.

Treated with observation and serial radiographs.

If painful biopsy to exclude other conditions


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Bone islandsBone islands


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ChondromaChondroma Benign lesions of hyaline cartilage. Phalanges of hand are most commonly affected. Usually asymptomatic rarely pathological fracture Enchondromas – arise from medullary canal,

Periosteal chondromas or Juxtacortical chondromas arise on surface

Multiple enchondromatosis – Ollier’s disease Multiple enchondromatosis + Soft tissue hemangiomas

= Maffuci syndrome Radiographically: benign appearing tumors with

intralesional calcification. Calcification is irregular and described as “strippled” “punctate” or “popcorn”. DD Chondrosarcoma

Treatment is observation with serial radiographs , curettage if it grows


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OsteochondromaOsteochondroma Bony mass with form of a stalk produced by

progressive endochondral ossification of growing cartilage.

Usually found on the metaphysis of long bones near physis.

Commonly distal femur, proximal tibia, and proximal humerus.

Clincally: pain, fracture, pressure symptoms like false aneurisms, neuropathies, palpable mass.

Multiple hereditary exostoses AD Two types: Pedunculated or Sessile Surgery(enbloc resection) is indicated when

unsightly, symptomatic or any suggestion of malignancy.


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Non Ossifying fibromaNon Ossifying fibroma Developmental abnormalities Metaphyseal region of long bones (40% distal

femur, 40% tibia, 10% fibula) 2 – 20 yrs Plain radiographs well lobulated lesion located

eccentrically in metaphysis. Asymptomatic


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Non Ossifying fibromaNon Ossifying fibroma


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Cortical DesmoidCortical Desmoid Is a irregularity in posteromedial aspect of

distal femoral metaphysis and usually is seen in boys 10 to 15 yr old.


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Benign fibrous Benign fibrous HistiocytomaHistiocytoma Diaphysis or epiphysis 30 – 40 yrs Radiographically it is well defined lytic,

expanding lesion with little periosteal reaction Extended curettage or wide resection


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Fibrous dysplasiaFibrous dysplasia Developmental anomaly Replacement of normal bone and marrow by

fibrous tissue and small, woven spicules of bone.

Radiographic appearance is lucent area with lucent area having a granular, ground-glass appearance

Surgical treatment is indicated when there is severe deformity, pathological fracture occurs or when significant pain exists.

Bisphosphonates treatment is probably beneficial.


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Fibrous dysplasiaFibrous dysplasia


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Osteofibrous DysplasiaOsteofibrous Dysplasia Ossifying fibroma of long bones Tibia and Fibula (middle third of Tibia) Diaphyseal Tibia is enlarged and often bowed anteriorly No pain unless pathological fracture Radiographs show eccentric intracortical

osteolysis with expansion of cortex


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Osteofibrous DysplasiaOsteofibrous Dysplasia


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Desmoplastic fibromaDesmoplastic fibroma Locally aggressive 2nd and 3rd decades Pain is complaint Radiographs show well circumscribed lytic

lesion with a narrow zone of transition and frequently a reactive bone.

Treatment wide resection


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Desomplastic fibromaDesomplastic fibroma


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Unicameral bone cystUnicameral bone cyst First 2 decades M:F is 2:1 Common in proximal humerus and femur, in

adults common in pelvis and calcaneum. Asymptomatic unless pathological fracture Radiographs centrally located , purely lytic

lesion with well marginated outline. Fallen fragment sign

Small, asymptomatic lesions treated with observation and serial radiographs

Large, symptomatic, and lesions in lower extremity are treated with curettage or aspiration and injection.


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Unicameral bone cystUnicameral bone cyst


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Aneurysmal bone cystAneurysmal bone cyst Locally destructive blood filled reactive lesions

of bone Proximal humerus, distal femur, proximal tibia

and spine < 20 yrs Mild to moderate pain, neurological symptoms

in spinal patient. Radiographically expansile lytic lesion that

elevates periosteum, but remains contained by a thin shell of cortical bone.

Treatment with extended curettage and grafting


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Aneurysmal bone cystAneurysmal bone cyst


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Intraosseous Ganglion Intraosseous Ganglion CystCyst Distal tibia, around knee and shoulder Intra osseous extensions of ganglia of local

soft tissues


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Epidermoid cystEpidermoid cyst Skull Radiographically they appear as rarefied

defects surrounded by sclerotic bone


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LipomaLipoma Asymptomatic


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HemangiomaHemangioma Vertebral bodies and

skull Radiographically in

spine it is characteristic with thickened, vertically oriented trabecuale giving the classic “jailhouse” appearance