Extraskeletal myxoid chondrosarcoma (EMC) is arare sarcoma thatgrows slowly but is hard to cure. It tends to spread and come back locally. The 2009 review by Drilon highlighted ineffectiveness of chemotherapy and emphasized an agressive approach to local control. In other words, the best chance to cure this disease is before it spreads. Radiotion is sometimes helpful but chemotherapy appears ineffective. There are , however, two case reports showing excellent responses to chemotherapy. Two patients are published to have responded well to Sutent. On the other hand, the one patient with EMC in a study of multiple non-GIST sarcomas had no response to sunitinib. The problem with basing therapy decisions on case reports is that there is a bias to publish only the positive cases; in this context, the single negative case is highly significant. Clearly more investigation is warranted before suntinib is used routinely for this admittedly rare cancer.
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