Cytoreductive surgery associated to hyperthermic intraperitoneal chemoperfusion for desmoplastic round small cell tumor with peritoneal carcinomatosis in young patients

Msika, Simon; Gruden, Enrico; Sarnacki, Sabine; Orbach, Daniel; Philippe-Chomette, Pascale; Castel, Benjamin; Sabaté, Jean-Marc; Flamant, Yves; Kianmanesh, Reza

doi:10.1016/j.jpedsurg.2010.03.002

« Previous Next »Journal of Pediatric Surgery
Volume 45, Issue 8 , Pages 1617-1621, August 2010

Cytoreductive surgery associated to hyperthermic intraperitoneal chemoperfusion for desmoplastic round small cell tumor with peritoneal carcinomatosis in young patients

Simon Msika
- Department of Digestive Surgery, University Paris 7 Denis Diderot, Hôpital Louis Mourier, Assistance Publique -Hôpitaux de Paris, 92700 Colombes, France
- Corresponding author.
Enrico Gruden
- Department of Digestive Surgery, University Paris 7 Denis Diderot, Hôpital Louis Mourier, Assistance Publique -Hôpitaux de Paris, 92700 Colombes, France
Sabine Sarnacki
- Department of Pediatric Surgery, University Paris Descartes, Hôpital Necker Enfants-Malades, Assistance Publique- Hôpitaux de Paris, 75006 Paris, France
Daniel Orbach
- Department of Pediatric Oncology, Institut Curie, 75005 Paris, France
Pascale Philippe-Chomette
- Department of Pediatric Surgery, University Paris 7 Denis Diderot, Hôpital Robert Debré, Assistance Publique -Hôpitaux de Paris, 75019 Paris, France
Benjamin Castel
- Department of Digestive Surgery, University Paris 7 Denis Diderot, Hôpital Louis Mourier, Assistance Publique -Hôpitaux de Paris, 92700 Colombes, France
Jean-Marc Sabaté
- Department of Digestive Oncology, University Paris 7 Denis Diderot, Hôpital Louis Mourier, Assistance Publique -Hôpitaux de Paris, 92700 Colombes, France
Yves Flamant
- Department of Digestive Surgery, University Paris 7 Denis Diderot, Hôpital Louis Mourier, Assistance Publique -Hôpitaux de Paris, 92700 Colombes, France
Reza Kianmanesh
- Department of Digestive Surgery, University Paris 7 Denis Diderot, Hôpital Louis Mourier, Assistance Publique -Hôpitaux de Paris, 92700 Colombes, France

Received 14 October 2009; received in revised form 2 March 2010; accepted 2 March 2010.

Abstract

Purpose

Desmoplastic 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.

Methods

Peritoneal 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.

Results

We 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.

Conclusions

Surgical 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.

Key words: Desmoplastic round small cell tumor, Peritoneal carcinomatosis, Hyperthermic intraperitoneal chemotherapy, Surgical cytoreduction, Young patients