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mesenchymal chondrosarcomaA rare high-grade chondrosarcoma consisting of undifferentiated mesenchymal cells admixed with cartilage.
Femur, ribs, jaw, skull, and spine are most common, though it may occur in any bone.
Pain, swelling. Two-thirds arise in bone—femur, ribs, spine, maxilla, mandible, pelvis and other tubular bones.
Comprise 2–10% of chondrosarcomas. Most common age 20 to 40; slight male predominance.
1–40-cm mass, pain, headache with intracranial lesions. Base of skull tumours present with brainstem symptoms and cranial nerve dysfunction.
Lytic lesions with stippled density characteristic of cartilaginous tumours; margin is ill-defined, but may be sharp if sclerosis is present. Expansile lesions are osteolytic. A lobulated (calcified “popcorn” lesion) extraosseous mass in soft tissues (e.g., skin, joint tissues, viscera, or meninges) may develop over time.
Lung, bone, viscera, lymph nodes.
Radical aggressive surgery. Complete excision yields a 27% survival; incomplete, 0%. Chemotherapy as for Ewing sarcoma.
Poor compared to other chondrosarcomas. Chemotherapy + complete surgical remission yields a 10-year disease-free survival of 72%, compared to 17% for surgery alone. They often recur and metastasise late > 10 years.