• Simon Msika, Enrico Gruden, Sabine Sarnacki, Daniel Orbach, Pascale Philippe-Chomette, Benjamin Castel, Jean-Marc Sabaté, Yves Flamant, and Reza Kianmanesh.
• Department of Digestive Surgery, University Paris 7 Denis Diderot, Hôpital Louis Mourier, Assistance Publique-Hôpitaux de Paris, 92700 Colombes, France. simon.msika@lmr.aphp.fr
• J. Pediatr. Surg. 2010 Aug 1;45(8):1617-21.

PurposeDesmoplastic round small cell tumor (DRSCT) is a rare intraabdominal mesenchymal tissue neoplasm in young patients and spreads through the abdominal cavity. Its prognosis is poor despite a multimodal therapy including chemotherapy, radiotherapy, and surgical cytoreduction (CS). hyperthermic intraperitoneal chemotherapy (HIPEC) is considered as an additional strategy in the treatment of peritoneal carcinomatosis; for this reason, we planned to treat selected cases of children with DRSCT using CS and HIPEC.MethodsPeritoneal disease extension was evaluated according to Gilly classification. Surgical cytoreduction was considered as completeness of cytoreduction-0 when no macroscopic nodule was residual; HIPEC was performed according to the open technique.ResultsWe described 3 cases: the 2 first cases were realized for palliative conditions and the last one was operated on with curative intent. There was no postoperative mortality. One patient was reoperated for a gallbladder perforation. There was no other complication related to HIPEC procedure.ConclusionsSurgical cytoreduction and HIPEC provide a local alternative approach to systemic chemotherapy in the control of microscopic peritoneal disease in DRSCT, with an acceptable morbidity, and may be considered as a potential beneficial adjuvant waiting for a more specific targeted therapy against the fusion protein.Copyright 2010 Elsevier Inc. All rights reserved.

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