Desmoplastic Small Round-cell Tumor: Retrospective Review of Institutional Data and Literature Review

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 Methods: Retrospective analysis of patients DSCRT diagnosis (2010-2020) was performed following Institutional Review Board approval. The follow-up period from pathological to the last patient contact. Endpoints were type response duration response. Results: In current analysis, first-line treatment all cases vincristine, anthracycline, cyclophosphamide alternating ifosfamide etoposide (VAC-IE) 100% a mean 9.8 (range=5-12) months. received 1-4 subsequent lines therapy. All temozolomide irinotecan (50% partial response, 8-9 months). Two that underwent consolidative cytoreductive surgery hyperthermic intraperitoneal chemotherapy had longer survival (30.6 vs. 11.2 suffered mortality median 17.8 (range=11.2-30.6) Conclusion: While multimodality always warranted DSCRT, options are by multicentric presentation, short-lived initial lack established therapy portending poor prognosis. Consolidative may improve survival.