TY - JOUR T1 - Desmoplastic Small Round-cell Tumor: Retrospective Review of Institutional Data and Literature Review JF - Anticancer Research JO - Anticancer Res SP - 3859 LP - 3866 DO - 10.21873/anticanres.15179 VL - 41 IS - 8 AU - THEJUS JAYAKRISHNAN AU - RYAN MOLL AU - ARIEL SANDHU AU - ANGELA SANGUINO AU - GURVEEN KAUR AU - SHIFENG MAO Y1 - 2021/08/01 UR - http://ar.iiarjournals.org/content/41/8/3859.abstract N2 - Background: Desmoplastic small round-cell tumor (DSCRT) in adults is an extremely rare (age-adjusted incidence 0.3 per million) and aggressive sarcoma with limited data for optimal management. Patients and Methods: Retrospective analysis of patients with DSCRT diagnosis (2010-2020) was performed following Institutional Review Board approval. The follow-up period was from pathological diagnosis to the last patient contact. Endpoints were type of response and duration of response. Results: In the current analysis, first-line treatment in all cases was vincristine, anthracycline, and cyclophosphamide alternating with ifosfamide and etoposide (VAC-IE) with 100% response for a mean duration of 9.8 (range=5-12) months. Patients received 1-4 subsequent lines of therapy. All patients received temozolomide with irinotecan (50% partial response, duration 8-9 months). Two patients that underwent consolidative cytoreductive surgery with hyperthermic intraperitoneal chemotherapy had a longer survival (30.6 vs. 11.2 months). Patients suffered 100% mortality with a median survival was 17.8 (range=11.2-30.6) months. Conclusion: While aggressive multimodality treatment is always warranted for DSCRT, the options are limited by the multicentric presentation, short-lived initial response and lack of established subsequent therapy portending a poor prognosis. Consolidative cytoreductive surgery following first-line therapy may improve survival. ER -