Mesenchymal Chondrosarcoma

To review the treatment and outcomes of patients with mesenchymal chondrosarcomas (MC).

Review of the pertinent literature.

MC is a rare aggressive small round blue cell malignancy that may arise in either bone or soft tissue. It usually presents in the 2^nd or 3^rd decade of life and exhibits an approximately equal gender predilection. Patients usually present with pain and swelling. The majority of MCs arise in either the trunk or extremities. Distant metastases are present at diagnosis in about 15% of patients. The most common sites for distant metastases are lung and bone. The optimal treatment is surgery. Although the role of adjuvant chemotherapy is unclear, an anthracycline-based chemotherapy regimen combined with ifosfamide or cisplatin, may be considered. Adjuvant radiation therapy (RT) is employed for patients with close (<5 mm) or positive margins as well as those with incompletely resectable tumors. The most common mechanism of recurrence is hematogenous dissemination. Although most recurrences are observed within 5 years of treatment, late recurrences are not unusual. The likelihood of successful salvage in the event of a recurrence is modest. The overall survival rates for all patients are approximately 50% at 5 years and 40% at 10 years. The overall survival rates for the subset of patients with localized disease that is resected are approximately 70% to 80% at 5 years and 60% at 10 years.

Patients with MCs are optimally treated with surgery. The role of adjuvant chemotherapy is uncertain. However, given the relatively high risk of recurrence, adjuvant chemotherapy should be considered in medically fit patients. Radiation therapy should be considered for those with incompletely resectable tumors and those with inadequate margins.