BACKGROUND No prospective trial with anthracycline-based chemotherapy has individually assessed response in a well-differentiated (WD)/dedifferentiated (DD) liposarcoma patient cohort. We conducted... Show moreBACKGROUND No prospective trial with anthracycline-based chemotherapy has individually assessed response in a well-differentiated (WD)/dedifferentiated (DD) liposarcoma patient cohort. We conducted a retrospective analysis of first-line chemotherapy in liposarcoma of intra-abdominal origin (IA-LPS) in patients who had entered the European Organisation for Research and Treatment of Cancer (EORTC)/Soft Tissue and Bone Sarcoma Group (STBSG) trials. METHODS We searched for all adult patients treated with first-line chemotherapy for advanced IA-LPS in the EORTC STBSG phase 2 and 3 trials from 1978. Treatment was aggregated into 5 groups: anthracycline alone, ifosfamide alone, doxorubicin plus ifosfamide (D+IFO), doxorubicin/cyclophosphamide/vincristine/dacarbazine, and "other" (brostallicin, trabectedin). Response was assessed prospectively by Response Evaluation Criteria in Solid Tumors or World Health Organization criteria. Progression-free survival (PFS) and overall survival (OS) were computed by Kaplan-Meier method. RESULTS A total of 109 patients with IA-LPS from 13 trials were identified (104 evaluable for response). Overall, there were 10/109 (9.2%) responders: 3/48 (6.3%) in the anthracycline alone group, 2/15 (13%) in the ifosfamide alone group, and 4/18 (22%) in the D+IFO group. At the 10-month median follow-up (interquartile range, 6-24), the median OS was 19 months (95% CI, 15-21) and median PFS 4 months (95% CI, 3-6). D+IFO achieved a not statistically significant longer median PFS (12 months) and median OS (31 months) than observed with other regimens. Univariate/multivariate analysis did not identify prognostic factors. CONCLUSIONS Cytotoxic chemotherapy, in particular anthracycline alone, had marginal activity in advanced IA-LPS. Ifosfamide-containing regimens showed higher activity, although it was not statistically significant and in a small number of cases, with the combination of doxorubicin and ifosfamide appearing to be the more active regimen available in fit patients. This series provides a benchmark for future trials on new drugs in WD/DD liposarcoma. Show less
Purpose of reviewRetroperitoneal sarcoma (RPS) is a rare disease, and until recently, its natural history and outcome were poorly understood. Recently, collaborations between individual centers... Show morePurpose of reviewRetroperitoneal sarcoma (RPS) is a rare disease, and until recently, its natural history and outcome were poorly understood. Recently, collaborations between individual centers have led to an unprecedented collection of retrospective and prospective data and successful recruitment to the first randomized trial as described here.Recent findingsA debate about the beneficial role of extended surgery in RPS triggered an initial collaboration between Europe and North America, the TransAtlantic RetroPeritoneal Sarcoma Working Group (TARPSWG). This collaboration has been instrumental in harmonizing the surgical approach among expert centers, characterizing the pattern of postresection failure of the different histological subtypes, identifying new ways to stage RPS and testing the role of preoperative radiotherapy in a randomized fashion (STRASS-1 study). The collaboration has now expanded to include centers from Asia, Australia and South America. A prospective registry has been started and a new randomized trial, STRASS-2, is in preparation to analyze the role of neoadjuvant chemotherapy for high-grade liposarcoma and leiomyosarcoma of the retroperitoneum.SummaryCollaboration is critical to study a rare disease like RPS. Both retrospective and prospective data are useful to improve knowledge, generate hypotheses and build evidence to test, whenever possible, in clinical trials. Show less
