Location: Trunk and craniofacial bones. Rare in the limbs.
Clinical: Pain and swelling.
Imaging: On x-ray – osteolytic lesion with permeative destruction of bone, ill-defined margins, breaking the cortex, with soft tissue mass, and with faded typical calcifications.
Histopathology: Dense round cells proliferation with hemangiopericytoma-like architecture. Within the tissue there are small foci or larger islands of well-differentiated hyaline cartilage.
Course and Staging: Fast growth, high rate of recurrence with inadequate surgery, and frequent metastases. Usually, IIB.
Treatment: Wide or radical resection. Chemotherapy is used but its real effectiveness is not yet known. Prognosis is poor.
Key Points
Clinical | Very rare in bone, adults, pain, and swelling |
Radiological | Aggressive osteolysis, with possible calcification |
Histological | Mixed population with isles of cartilagine and undifferentiated round cells |
Differential diagnosis | None
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