The clinical approach to desmoplastic small round cell tumor

Background and Objectives: Desmoplastic small round cell tumor (DSRCT) is a n increasingly recognized entity with a historically poor prognosis. This a rticle describes the present clinical management of these patients, Methods : We retrospectively reviewed our experience with 40 histologically proven cases of desmoplastic small round cell tumor diagnosed between 7/1/72 and 1 0/1/99. Thirty-five of these patients are the subjects of a previous report . Demographic data, mode of presentation, completeness of resection, and ov erall survival were assessed. Results: The overall survival from DSRCT rema ins very poor and in our series was 29% at three years from diagnosis. Ther e is a significant correlation between use of intense alkylator therapy (P6 protocol), and gross total resection with improved overall survival. It is often technically feasible to remove large peritoneal masses using a tange ntial dissection technique. Extensive hepatic parenchymal or hilar involvem ent or extensive infiltration of the diaphragm in the region of the septum transversum and hepatic veins usually makes gross total resection impossibl e with a corresponding worsened prognosis. Conclusion: We recommend inducti on chemotherapy (P6) followed by aggressive surgical debulking and external beam radiotherapy for the treatment of DSRCT. Further progress will requir e new forms of treatment. (C) 2000 Published by Elsevier Science Ltd.