Osteosarcoma

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Osteosarcoma : 

Osteosarcoma Dr. Varun

PowerPoint Presentation: 

Dr. Varun

Intro – Central osteosarcoma : 

Intro – Central osteosarcoma 2 nd MC primary malignant tumor of bone (20%) Forms neoplastic osteoid 75% cases 10-25 yrs (median 17-18) M:F = 2:1 ( Avg age on onset earlier in female) Dr. Varun

Clinical features: 

Clinical features Painful swelling at site of lesion Insidious, transitory -> severe & persistent h/o antecedent trauma – traumatic determinism Systemic features – unusual Interval btw onset of symptoms & recognition of tumor - ≥6 months Sometimes a/w acute DVT – tumor thrombus Dr. Varun

Location: 

Location Metaphyseal abutting physes Calvaria , sacrum, pelvis, mandible, maxilla, scapula, clavicle, ribs, calcaneus , & spine Dr. Varun 41.5% 16% 15%

Pathology : 

Pathology Cause unknown Factors associated Beryllium, viruses, radiation exposure, Paget’s, electrical burns, trauma Grows in 3 patterns Osseous neoplastic tissue - Sclerotic lesion 50% Sarcomatous connective tissue - Lytic lesion 25% Moderate osteoid matrix – mixed pattern 25% Dr. Varun

Metastases : 

Metastases Hematogenous route Lungs (95%) Canon ball mets Hypertrophic osteoarthropathy Spontaneous pneumothorax – subpleural nodules rupture into pleural space Bones 50% Kidneys 12% Dr. Varun

Radiologic features: 

Radiologic features Metaphyses 75% of long bones Epiphyseal plate functions as barrier to tumor migration Classically Focal metaphyseal lesion Mottled, permeative Poor zone of transition Dense ivory or sclerotic region involving medulla Cortical disruption Irregular periosteal new bone formation Often takes place within an extracortical , dense soft tissue mass that displays transverse spicules or radiating striations (sunburst or sunray) Elevated periosteum by tumor tissue on upper and lower margins of the lesion ( Codmans reactive triangles) Dr. Varun

Codman’s triangle: 

Codman’s triangle Reactive repsonse to elevation of periosteum D/Ds Traumatic periostitis Osteomyelitis Eosinophilic granuloma Thyroid acropachy Dr. Varun

Radiologic features: 

Radiologic features Bone expansion Pathologic fracture following trauma Sclerotic lesion develops a roughened lobulated margin – cumulus cloud appearance Soft tissue mass formation – common Ossification within is frequent CT/MR Marrow infiltration, soft tissue mass, response to therapy Dr. Varun

Treatment, prognosis: 

Treatment, prognosis Sclerotic – slower, less aggressive clinical course Lytic – rapidly fatal Average time between recognition of pulmonary mets & death ~ 6 months >19yrs, large size, pathologic #s – poor prognosis Jaw tumors, bone surface tumors – better prognosis Dr. Varun

PowerPoint Presentation: 

Dr. Varun

PowerPoint Presentation: 

Dr. Varun

Mutlicentric osteosarcoma: 

Mutlicentric osteosarcoma Sclerosing osteogenic sarcomatosis , osteosarcomatosis , childhood multifocal osteosarcoma Approx 20 cases have only been reported! ALP elevated – blastic type 5-10 yrs Rapid and fatal Early lung mets Dr. Varun

Radiologic features: 

Radiologic features Dramatic Metaphysis of long bones, no specific sites, also involves flat bones Radio opaque lesions – bilaterally symmetric Initially resembles bone islands, later fill the entire medullary space Dr. Varun

D/D: 

D/D Heavy metal poisoning – lead Metaphyseal increase in density Oval/round – multifocal osteoCa Transverse, band like – metal poisoning Engelmann’s disease ( diaphyseal sclerosis) Melorheostosis Osteopetrosis Osteopoikilosis Dr. Varun

PowerPoint Presentation: 

Dr. Varun

Parosteal osteosarcoma: 

Parosteal osteosarcoma Juxtacortical / paraosteal /surface osteosarcoma Arises in juxta cortical location within periosteum 3-4% of all osteosarcomas Grows slowly Swelling or mass with dull aching pain for a long duration Dr. Varun

Incidence, location, pathology: 

Incidence, location, pathology 30-50yrs, M=F Posterior surface of distal femoral epiphyses 50% Proximal tibia & humerus 25% Ulna, radius, clavicle, metacarpals, phalanges, mandible Large, average 10cm size. Lobulated , sessile, bony hard mass, having an intimate broad based attachment to underlying bone Occasionally a periosteal fibrous tissue layer separates tumor from the cortical surface – cleavage plane Important to identify if extending into medullary canal Dr. Varun

Radiologic features: 

Radiologic features Dense homogeneous juxtacortical mass Classically involves popliteal surface of distal femur Radiolucent cleft separates majority of mass from cortex of the femur (30%) Cleft 1-3mm in width, stops abruptly at stalk of the tumor Aka cleavage plane or string sign Lobular outline, no periosteal new bone formation noted Dr. Varun

Radiologic D/D : 

Radiologic D/D Post traumatic myositis ossificans Myositis usually clearly separated from bone Inner portion of sarcomas uniformly dense, with less dense peripheral margin Myositis – less dense centrally with a halo like rim of peripheral cortical bone Serial films – myositis shrinks, sarcomas grow Dr. Varun

Prognosis : 

Prognosis High recurrence rate following en bloc excision 50% 5 year survival rate 70% following excision Dr. Varun

PowerPoint Presentation: 

Dr. Varun

Secondary osteosarcoma: 

Secondary osteosarcoma Malignant degeneration of benign disorders Paget’s, polyostotic fibrous dysplasia, hereditary multiple exostosis , enchondromatosis Ionizing radiation – latent period 15 years Thorotrast injection Radiologically indistinguishable from primary Dr. Varun

PowerPoint Presentation: 

Paget’s Dr. Varun

Extraosseous osteosarcoma: 

Extraosseous osteosarcoma Originate in an extra osseous site Soft tissues of thigh, pleura, heart valves, dura , retroperitoneum , buttock, axilla , breast, renal capsule 30-50 yrs Lung metastases Histologically similar to osseous osteosarcoma Large soft tissue mass adjacent to a bone. Non specific, bone formation within it may lead to a proper diagnosis Dr. Varun