Chordomas are tumors originating from embryonic remnants of the primitive notochord. Metastatic spread is observed in 7-14% of patients and imatinib mesylate exhibits antitumor activity in some reports of patients with chordoma. Obviously, for a rare cancer like chordoma, there is not muche experi3nce with chemotherapy. Some patents have been reported to have received and responded to gemcitabine or navelbine. There are no drugs currently approved to treat chordoma, however a clinical trial conducted in Italy using imatinib demonstrated a modest response in some chordoma patients. The same group in Italy found that the combination of imatinib and sirolimus caused a response in several patients whose tumors progressed on imatinib alone. A phase II study has been was published in 2007. In it: In 44 pts evaluable for antitumor response, 37 (84%) had stable disease as their best RECIST response, which was maintained for more than 6 months in 32, for a clinical benefit rate (CR+PR+SD=6mos) of 73%. In 7 of these SD pts (16%), a degree of objective tumor shrinkage was reported. Centralized. On ITT analysis, median PFS was 32 wks, with 38% of pts free from progression at one year, and 16% on treatment at 18 months.
S. Stacchiotti, S. Ferrari, V. Ferraresi, G. Grignani, F. Crippa, A. Messina, C. Spreafico, E. Tamborini, A. Gronchi, P. G. Casali Imatinib mesylate in advanced chordoma: A multicenter phase II study. Journal of Clinical Oncology, 2007 ASCO Annual Meeting Proceedings Part I. Vol 25, No. 18S (June 20 Supplement), 2007: 10003
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