Treatment of metastatic Extraskeletal myxoid chondrosarcoma (EMC – pro
Extraskeletal myxoid chondrosarcoma (EMC) is a rare soft tissue sarcoma, marked by NR4A3 rearrangement. Most chondrosarcomas (with the exception of mesenchymal chondrosarcoma) do not respond to chemotherapy or radiation therapy. This makes surgical management crucial in the treatment of chondrosarcoma. Chemotherapy or radiation therapy is sometimes considered for patients with recurrent, metastatic or dedifferentiated disease. Unfortunately, these treatments have not been proven to be effective, and there is no standard protocol to follow. Sutent has shown some activity in this rare condition. However, the reports are preliminary and with small series of patients. For example, Stachiotti et al treated 10 patients. Six patients had a Response Evaluation Criteria in Solid Tumours (RECIST) partial response (PR), two were stable, two progressed. ANotehr recently reported drug is Adriamycin.
Stacchiotti S et al, Activity of sunitinib in extraskeletal myxoid chondrosarcoma. Eur J Cancer. 2014 Jun;50(9):1657-64.
Silvia Stacchiotti et al, Anthracycline-based chemotherapy in extraskeletal myxoid chondrosarcoma: a retrospective study. Clin Sarcoma Res. 2013; 3: 16