Extraskeletal myxoid chondrosarcoma (EMC) is a genetically distinct and rare sarcoma that tends to be indolent but to eventually recurr locally and metastastzise. The 2009 review by Drilon highlighted ineffectiveness of chemotherapy and emphasized an agressive approach to local control. There are , however, two case reports showing excellent responses to chemotherapy. Radiotherapy is sometimes helpful. Sutent was effective in two cases reported by Stacchiotti et al. On the other hand, the one patient with EMC in a study of multiple non-GIST sarcomas had no response to sunitinib(George et al). 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 study is warranted before suntinib is used routinely for this admittedly rare cancer.
Drilon AD, Popat S, Bhuchar G, D’Adamo DR, Keohan ML, Fisher C, Antonescu CR, Singer S, Brennan MF, Judson I, Maki RG. Extraskeletal myxoid chondrosarcoma: a retrospective review from 2 referral centers emphasizing long-term outcomes with surgery and chemotherapy. Cancer. 2008 Dec 15;113(12):3364-71.
Kun Han, Yuan-Jue Sun, Zan Shen, Jian-jun Zhang, Feng Lin, Hui Zhao, Saleem Meerani, Yang Yao
Extraskeletal myxoid chondrosarcoma: a case report of complete remission by chemotherapy and review of the literature. BMJ Case Reports 2010; doi:10.1136/bcr.07.2009.2128
Suzanne George, Priscilla Merriam, Robert G. Maki, Annick D. Van den Abbeele, Jeffrey T. Yap, Timothy Akhurst, David C. Harmon, Gauri Bhuchar, Margaret M. O’Mara, David R. D’Adamo, Jeffrey Morgan, Gary K. Schwartz, Andrew J. Wagner, James E. Butrynski, George D. Demetri, and Mary L. Keohan, Multicenter Phase II Trial of Sunitinib in the Treatment of Nongastrointestinal Stromal Tumor Sarcomas, JCO VOLUME 27 NUMBER 19 JULY 1 2009
K, Fujiwara T, Beppu Y, Chuman H, Yoshida A, Kawano H, Kawai A; Extraskeletal myxoid chondrosarcoma: a review of 23 patients treated at a single referral center with long-term follow-up; Ogura Archives of Orthopaedic and Trauma Surgery (Jun 2012), http://www.docguide.com/extraskeletal-myxoid-chondrosarcoma-review-23-patients-treated-single-referral-center-long-term-foll?tsid=5
Stacchiotti S, Dagrada GP, Morosi C, Negri T, Romanini A, Pilotti S, Gronchi A, Casali PG.
Extraskeletal myxoid chondrosarcoma: tumor response to sunitinib.Clin Sarcoma Res. 2012 Oct 11;2(1):22.
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