Mesenchymal chondrosarcoma: clinicopathologic study of 20 cases.

CONTEXT: Mesenchymal chondrosarcoma is a rare, high-grade malignancy of bone or soft tissue with a unique, biphasic histology and poor prognosis. Because of its rarity and variable length of disease-free survival, the natural history of the disease remains poorly understood.
OBJECTIVE: To present clinical, radiographic, and histopathologic features of mesenchymal chondrosarcoma from one of the largest case series collected by a single, senior-level bone pathologist.
DESIGN: Twenty cases were reviewed in consultations spanning 45 years.
RESULTS: Eighteen tumors (90%) originated in bone, and 2 tumors (10%) were of extraskeletal origin. Of the skeletal tumors, locations included craniofacial bones (n  =  9; 50%), ribs and chest wall (n  =  4; 22%), sacrum and spinal elements (n  =  3; 17%), and lower extremities (n  =  2; 11%), whereas soft tissue tumors were located about the scapula (n  =  1; 50%) and lower extremity (n  =  1; 50%). Plain radiographs demonstrated calcified, osteolytic lesions with extraosseous extension. Typical histologic features were identified consisting of small, round or spindled cells, interspersed with hyaline cartilage islands. Seventeen patients (85%) were treated surgically, and 8 patients (40%) received adjuvant treatment. Seven patients (35%) were living at last follow-up, 1.8 to 12.5 years after diagnosis, and 8 patients (40%) died between 1.2 and 21.8 years after diagnosis.
CONCLUSIONS: Mesenchymal chondrosarcoma presents multiple challenges. Diagnostic pitfalls include inadequate biopsy samples, which may result in sample error. Sox9 has been proposed as a unique marker for mesenchymal chondrosarcoma which may improve diagnostic specificity. Treatment and prognosis vary considerably. Patients who receive surgery and chemotherapy seem to fare better. Multicenter studies with higher sample numbers may improve our understanding of this malignancy.