151. Cytoreductive surgery associated to hyperthermic intraperitoneal chemoperfusion for desmoplastic round small cell tumor with peritoneal carcinomatosis in young patients
- Author
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Simon Msika, Pascale Philippe-Chomette, Daniel Orbach, Yves Flamant, Enrico Gruden, Benjamin Castel, Reza Kianmanesh, Sabine Sarnacki, and Jean-Marc Sabate
- Subjects
Male ,medicine.medical_specialty ,Adolescent ,medicine.medical_treatment ,Abdominal cavity ,Adenocarcinoma ,Gallbladder perforation ,Disease-Free Survival ,Antineoplastic Combined Chemotherapy Protocols ,medicine ,Humans ,Carcinoma, Small Cell ,Peritoneal Neoplasms ,Chemotherapy ,business.industry ,Carcinoma ,Palliative Care ,Multimodal therapy ,General Medicine ,Hyperthermia, Induced ,Combined Modality Therapy ,Surgery ,Peritoneal carcinomatosis ,Tumor Burden ,Radiation therapy ,medicine.anatomical_structure ,Treatment Outcome ,Chemotherapy, Cancer, Regional Perfusion ,Pediatrics, Perinatology and Child Health ,Hyperthermic intraperitoneal chemotherapy ,Female ,Peritoneum ,Complication ,business ,Injections, Intraperitoneal - Abstract
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.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.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.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.
- Published
- 2009