Clear cell sarcoma is a rare translocation-related
sarcoma. There have been few studies documenting the response rate and progression-free survival in
clear cell sarcoma patients treated with palliative
chemotherapy. The prospectively maintained databases of two referral centres were searched to identify
clear cell sarcoma patients treated with
chemotherapy. Twenty-four patients were treated with palliative first-line
chemotherapy with a median age of 30 years at diagnosis. There were 18 men and 6 women. One (4%) achieved a partial response and 9 (38%) had stable disease. Fourteen patients (58%) progressed on
therapy. The median progression-free survival was 11 weeks (95% CI, 3–20 weeks). The median overall survival from commencing first-line
chemotherapy was 39 weeks (95% CI, 34–45 weeks). Second-line
chemotherapy was administered to 12 patients, 11 (92%) of these progressed and one (8%) had stable disease. Of the 5 patients treated with third-line
chemotherapy, 4 (80%) progressed and one (20%) had stable disease. One patient received fourth-line
chemotherapy and maintained stable disease for 4 months. Conventional
chemotherapy has minimal activity in
clear cell sarcoma as documented by the response rate of 4% and median progression-free survival of 11 weeks in this retrospective series. These data provide a reference for response and outcome in the assessment of novel agents in this histological subtype.