mesenchymal chondrosarcoma


mesenchymal chondrosarcoma

A rare high-grade chondrosarcoma consisting of undifferentiated mesenchymal cells admixed with cartilage.
 
Sites
Femur, ribs, jaw, skull, and spine are most common, though it may occur in any bone.
Clinical findings
Pain, swelling. Two-thirds arise in bone—femur, ribs, spine, maxilla, mandible, pelvis and other tubular bones.
Demographics
Comprise 2–10% of chondrosarcomas. Most common age 20 to 40; slight male predominance.
Clinical findings
1–40-cm mass, pain, headache with intracranial lesions. Base of skull tumours present with brainstem symptoms and cranial nerve dysfunction.
Imaging
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.
Metastatic sites
Lung, bone, viscera, lymph nodes.
Management
Radical aggressive surgery. Complete excision yields a 27% survival; incomplete, 0%. Chemotherapy as for Ewing sarcoma.
Prognosis
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.