title: Cytoreductive surgery associated to hyperthermic intraperitoneal chemoperfusion for desmoplastic round small cell tumor with peritoneal carcinomatosis in young patients.
abstract: OBJECTIVE
  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.
authors: 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
authors: Simon
authors: S
authors: Msika
authors: Enrico
authors: E
authors: Gruden
authors: Sabine
authors: S
authors: Sarnacki
authors: Daniel
authors: D
authors: Orbach
authors: Pascale
authors: P
authors: Philippe-Chomette
authors: Benjamin
authors: B
authors: Castel
authors: Jean-Marc
authors: JM
authors: Sabaté
authors: Yves
authors: Y
authors: Flamant
authors: Reza
authors: R
authors: Kianmanesh
mesh_terms: D000230:Adenocarcinoma / Q000188:drug therapy / Q000473:pathology / Q000601:surgery; D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002277:Carcinoma / Q000188:drug therapy* / Q000473:pathology / Q000601:surgery*; D018288:Carcinoma, Small Cell / Q000188:drug therapy / Q000601:surgery; D010478:Chemotherapy, Cancer, Regional Perfusion / Q000379:methods*; D003131:Combined Modality Therapy; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D006979:Hyperthermia, Induced / Q000379:methods*; D007274:Injections, Intraperitoneal; D008297:Male; D010166:Palliative Care; D010534:Peritoneal Neoplasms / Q000188:drug therapy* / Q000473:pathology / Q000556:secondary / Q000601:surgery*; D010537:Peritoneum / Q000473:pathology / Q000601:surgery; D016896:Treatment Outcome; D047368:Tumor Burden / Q000187:drug effects