Efficacy of trabectedin in malignant solitary fibrous tumors: A retrospective analysis from the French Sarcoma Group

Abstract

Background: Advanced malignant solitary fibrous tumors (SFTs) are rare soft-tissue sarcomas with a poor prognosis. Several treatment options have been reported, but with uncertain rates of efficacy. Our aim is to describe the activity of trabectedin in a retrospective, multi-center French series of patients with SFTs. Methods: Patients were mainly identified through the French RetrospectYon database and were treated between January 2008 and May 2013. Trabectedin was administered at an initial dose of 1.5 mg/m2, q3 weeks. The best tumor response was assessed according to the Response Evaluation Criteria In Solid Tumors 1.1. The Kaplan-Meier method was used to estimate median progression-free survival (PFS) and overall survival (OS). The growth-modulation index (GMI) was defined as the ratio between the time to progression with trabectedin (TTPn) and the TTP with the immediately prior line of treatment (TTPn-1). Results: Eleven patients treated with trabectedin for advanced SFT were identified. Trabectedin had been used as second-line treatment in 8 patients (72.7%) and as at least third-line therapy in a further 3 (27.3%). The best RECIST response was a partial response (PR) in one patient (9.1%) and stable disease (SD) in eight patients (72.7%). Disease-control rate (DCR=PR+SD) was 81.8%. After a median follow-up of 29.2months, the median PFS was 11.6months (95 % CI=2.0; 15.2months) and the median OS was 22.3months (95 % CI=9.1months; not reached). The median GMI was 1.49 (range: 0.11-4.12). Conclusion: Trabectedin is a very promising treatment for advanced SFTs. Further investigations are needed.