37-y.o male with HME and knee pain. Axial T1, T2 and STIR MRI slices at popliteal region. Large cartilaginous cap and possible compression of the popliteal artery by osteochondroma. MRA was performed to evaluate popliteal A. pseudoaneurysm (large arrow). Pathology specimen obtained from the cartilagenous cap showed increased cellularity suggestive of malignant degeneration. Operative care was planned
Ewing sarcoma: age: 2-20, uncommon in black patients. 2nd m/c highly malignant bone neoplasm in children that typically arises from
medullary cavity (Round cell tumors). Key symptom: bone pain that may mimic infection (ESR/CRP/WBC) Considered PNET
Key Rad Dx: aggressive moth-eaten/permeative lucent lesions in the shaft of long bones with large soft tissue invasion/typical onion skin
periostitis. May produce saucerisation
May affect flat bones. May appear as sclerotic in 33%. Early lung mets (25-30%) bone-to-bone mets
Poor prognosis if delayed Dx. Imaging steps: 1st step x-rad, MRI is v. important followed by biopsy. CXR/CT PET-CT
Rx: combined rad-chemo, operative.
Malignant fibrous histiocytoma (MFH) reclassified as Pleomorphic Undifferentiated Sarcoma (PUS) is the m/c S.T. sarcoma. MFH is
aggressive biologically with poor prognosis
M>F (1.2:1) 30-80 with peak in a 6th decade. 25-40% of all adults sarcomas m/c extremities. Retroperitoneum next (worst prognosis d/t late Dx and large growth w/o symptoms)
Clinically: painful, hard mass typically about the knee or thigh. Histology: poorly differentiated/undifferentiated malignant fibroblasts, myofibroblasts and other mesenchymal cells
Imaging: MRI is the modality of choice with T1, T2, T1+C. Typically appears as aggressive heterogeneous mass intermediate to low signal on T1 and high signal on T2 with areas of necrosis and enhancement on T1+C. May
appear misleadingly encapsulated w/o true capsule
Management: operative with radiation and chemotherapy. Tumour depth is crucial for prognosis. 80% 5-year survival if <5cm deep in ST and 50% if >5-cm deep in ST.
Synovial sarcoma: common malignant ST neoplasm esp. in younger patients or older children/adolescents. M/C found in knee area
Clinically: can present slowly as palpable mass in the extremity often ignored d/t slow growth
Imaging is the key: radiography may reveal ST. density/mass. Some synovial sarcomas may show calcification and mistaken for Myositis Ossificanse or heterotopic bone formation
MRI with T1,T2 and T1+C are Dx modality of choice. Other modalities: US, CT are non-specific
Management: operative, chemo-radiation
Prognosis: variable depending on size, invasion, metastasis
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