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Sarcoma
Volume 2012 (2012), Article ID 714986, 5 pages
http://dx.doi.org/10.1155/2012/714986
Review Article

Desmoplastic Small Round Cell Tumor: Current Management and Recent Findings

1Department of Immunity, Virus and Microenvironnement, Cancer Research Center of Lyon (CRCL), UMR INSERM 1052—CNRS 5286, Leon Berard Cancer Center, 28 Rue Laennec, 69008 Lyon, France
2Medical Oncology Department, Leon Berard Cancer Center, 28 Rue Laennec, 69008 Lyon, France

Received 14 December 2011; Accepted 22 January 2012

Academic Editor: Isabelle Ray-Coquard

Copyright © 2012 Armelle Dufresne et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Desmoplastic small round cell tumor (DSRCT) is a rare and highly aggressive mesenchymal tumor that develops in the abdominal cavity of young men adults. Patients typically present with symptoms of abdominal sarcomatosis. Diagnosis is based on histological analysis of biopsies which typically show small round blue cells in nests separated by an abundant desmoplastic stroma. DSRCT is associated with a unique chromosomal translocation t(11:22) (p 13; q 12) that involves the EWSR1 and WT1 genes. The prognosis is particularly poor; median survival ranges from 17 to 25 months, largely due to the presentation of the majority of patients with metastatic disease. Management of DSRCT remains challenging and current schemes lack a significant cure rate despite the use of aggressive treatments such as polychemotherapy, debulking surgery and whole abdominal radiation. Several methods are being evaluated to improve survival: addition of chemotherapy and targeted therapies to standard neoadjuvant protocol, completion of surgical resection with HIPEC, postoperative IMRT, treatment of hepatic metastases with [90Y]Yttrium microsphere liver embolization.