Systemic therapy in clear cell sarcoma.

Abstract

10098 Background: Clear cell sarcoma is a rare soft tissue sarcoma subtype associated with the characteristic translocation t(12;22)(q13;q12). There have been few studies documenting the response rate and progression-free survival in clear cell sarcoma patients treated with palliative chemotherapy. Methods: The prospectively maintained databases of two referral centres were searched to identify clear cell sarcoma patients treated with chemotherapy. Results: Twenty-four patients were treated with palliative first-line chemotherapy with a median age of 30 years at diagnosis. All patients underwent surgical resection of the primary tumour as initial management. At the time of analysis 22 patients had died and 2 were lost to follow-up. There were 18 males and 6 females. Two of these patients were treated with neoadjuvant chemotherapy at the time of initial diagnosis and received palliative systemic therapy on progression. One of these achieved a partial response and the response of the other to neoadjuvant therapy was not known. For the patients receiving chemotherapy for recurrent/metastatic disease (n=24), one (4%) achieved a partial response and 9 (37%) 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 that received fourth-line chemotherapy maintained stable disease for 4 months. Conclusions: 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. No significant financial relationships to disclose.