TY - JOUR U1 - Wissenschaftlicher Artikel A1 - Pink, Daniel A1 - Bertz-Lepel, Janina A1 - Busemann, Christoph A1 - Bitz, Ulrich A1 - Reichardt, Peter T1 - Efficacy of Trabectedin in Patients with Advanced or Metastatic Alveolar Soft-Part Sarcoma JF - Oncology Research and Treatment N2 - Background: Alveolar soft-part sarcoma (ASPS) is a rare sarcoma often occurring in young patients that is characterized by the unbalanced translocation der(17)t(X;17) (p11;q25). Although itusuallyshowsan indolent clinical course, the prognosis is usually poor in advanced disease. Since standard chemotherapy regimens used in soft-tissue sarcomas lack efficacy in ASPS, new therapeutic options are needed. We investigated the efficacy of trabectedin, which has demonstrated activity in a variety of cancer types including some of the most prevalent translocation-related sarcomas. Patients and Methods: 7 patients with metastatic or advanced ASPS treated with trabectedin in the Sarcoma Center Berlin-Brandenburg and the University Hospital of Greifswald were analyzed for median progression-free survival (mPFS), overall survival (OS), and therapy-related toxicity. Results: In 6 patients with documented disease progression, disease stabilization was reached with trabectedin; only 1 patient experienced progressive disease. The mPFS and OS were 7 months and 21 months, respectively, since the start of trabectedin treatment. Overall, no severe Common Toxicity Criteria (CTC) grade 3 or 4 toxicity was observed. Conclusions: The poor prognosis of patients with ASPS has so far been due to the unavailability of effective systemic treatments. Trabectedin can be considered the only currently registered drug with clinical activity in this disease. KW - - KW - Trabectedin KW - ASPS Y1 - 2012 UN - https://nbn-resolving.org/urn:nbn:de:gbv:9-opus-31042 UR - www.karger.com/onk SN - 2296-5270 SS - 2296-5270 SN - 2296-5262 SS - 2296-5262 U6 - https://doi.org/10.1159/000338342 DO - https://doi.org/10.1159/000338342 PM - 22868503 VL - 35 IS - 5 SP - 249 EP - 252 PB - S. Karger AG CY - Basel, Switzerland ER -