title: Abdominal desmoplastic small round cell tumor without extraperitoneal metastases: Is there a benefit for HIPEC after macroscopically complete cytoreductive surgery?
abstract: BACKGROUND
  Desmoplastic Small Round Cell Tumor (DSRCT) is a rare disease affecting predominantly children and young adults and for which the benefit of hyperthermic intraperitoneal chemotherapy (HIPEC) after complete cytoreductive surgery (CCRS) remains unknown.
  
  
  METHODS
  To identify patients with DSRCT without extraperitoneal metastases (EPM) who underwent CCRS between 1991 and 2015, a retrospective nation-wide survey was conducted by crossing the prospective and retrospective databases of the French Network for Rare Peritoneal Malignancies, French Reference Network in Sarcoma Pathology, French Sarcoma Clinical Network and French Pediatric Cancer Society.
  
  
  RESULTS
  Among the 107 patients with DSRCT, 48 had no EPM and underwent CCRS. The median peritoneal cancer index (PCI) was 9 (range: 2-27). Among these 48 patients, 38 (79%) had pre- and/or postoperative chemotherapy and 23 (48%) postoperative whole abdominopelvic radiotherapy (WAP-RT). Intraperitoneal chemotherapy was administered to 11 patients (23%): two received early postoperative intraperitoneal chemotherapy (EPIC) and nine HIPEC. After a median follow-up of 30 months, the median overall survival (OS) of the entire cohort was 42 months. The 2-y and 5-y OS were 72% and 19%. The 2-y and 5-y disease-free survival (DFS) were 30% and 12%. WAP-RT was the only variable associated with longer peritoneal recurrence-free survival and DFS after CCRS. The influence of HIPEC/EPIC on OS and DFS was not statistically conclusive.
  
  
  CONCLUSIONS
  The benefit of HIPEC is still unknown and should be evaluated in a prospective trial. The value of postoperative WAP-RT seems to be confirmed.
authors.affiliation: Department of Surgical Oncology, Gustave Roussy Cancer Campus, Villejuif, France.
authors.forename: C
authors.initials: C
authors.lastname: Honoré
authors.affiliation: Department of Radiotherapy, Bergonié Institute, Bordeaux, France.
authors.forename: V
authors.initials: V
authors.lastname: Atallah
authors.affiliation: Department of Medical Oncology, Gustave Roussy Cancer Campus, Villejuif, France.
authors.forename: O
authors.initials: O
authors.lastname: Mir
authors.affiliation: Department of Pediatric, Adolescent and Young Adult Oncology, Curie Institute, Paris, France.
authors.forename: D
authors.initials: D
authors.lastname: Orbach
authors.affiliation: Department of Surgical Oncology, Claudius Régaud Institute, Toulouse, France.
authors.forename: G
authors.initials: G
authors.lastname: Ferron
authors.affiliation: Department of Radiotherapy, Gustave Roussy Cancer Campus, Villejuif, France.
authors.forename: C
authors.initials: C
authors.lastname: LePéchoux
authors.affiliation: Department of General and Digestive Surgery, Hautepierre Hospital, Strasbourg University, Strasbourg, France.
authors.forename: J B
authors.initials: JB
authors.lastname: Delhorme
authors.affiliation: Department of Pediatric Surgery, Robert Debré Hospital, Paris, France.
authors.forename: P
authors.initials: P
authors.lastname: Philippe-Chomette
authors.affiliation: Department of Pediatric Surgery, Necker Enfants Malades Hospital, Paris Descartes University, Paris, France.
authors.forename: S
authors.initials: S
authors.lastname: Sarnacki
authors.affiliation: of Digestive Surgery, Louis Mourier Hospital, Paris Diderot University, Colombes, France.
authors.forename: S
authors.initials: S
authors.lastname: Msika
authors.affiliation: Department of Pathology, Gustave Roussy Cancer Campus, Villejuif, France.
authors.forename: P
authors.initials: P
authors.lastname: Terrier
authors.affiliation: Department of Surgical Oncology, Lyon Civil Hospices, South Lyon University Hospital Center, Lyon, France.
authors.forename: O
authors.initials: O
authors.lastname: Glehen
authors.affiliation: Department of Pediatric Surgery, Kremlin-Bicêtre Hospital, Paris 11 University, Kremlin-Bicêtre, France.
authors.forename: H
authors.initials: H
authors.lastname: Martelli
authors.affiliation: Department of Pediatric Oncology, Gustave Roussy Cancer Campus, Villejuif, France.
authors.forename: V
authors.initials: V
authors.lastname: Minard-Colin
authors.affiliation: Department of Medical Oncology, Paoli-Calmettes Institute, Aix-Marseille University, Marseille, France.
authors.forename: F
authors.initials: F
authors.lastname: Bertucci
authors.affiliation: Department of Medical Oncology, Leon Bérard Center, Lyon, France.
authors.forename: J Y
authors.initials: JY
authors.lastname: Blay
authors.affiliation: Department of Surgery, Curie Institute, Paris, France.
authors.forename: S
authors.initials: S
authors.lastname: Bonvalot
authors.affiliation: Department of Surgical Oncology, Gustave Roussy Cancer Campus, Villejuif, France.
authors.forename: D
authors.initials: D
authors.lastname: Elias
authors.affiliation: Department of Medical Oncology, Gustave Roussy Cancer Campus, Villejuif, France.
authors.forename: A
authors.initials: A
authors.lastname: LeCesne
authors.affiliation: Department of Radiotherapy, Bergonié Institute, Bordeaux, France.
authors.forename: P
authors.initials: P
authors.lastname: Sargos
mesh_terms: D000293:Adolescent; D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D065426:Cytoreduction Surgical Procedures; D058405:Desmoplastic Small Round Cell Tumor / Q000401:mortality / Q000473:pathology / Q000601:surgery / Q000628:therapy*; D004317:Doxorubicin / Q000627:therapeutic use; D005260:Female; D005720:Gamma Rays / Q000627:therapeutic use; D006801:Humans; D006979:Hyperthermia, Induced / Q000379:methods*; D007069:Ifosfamide / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D009367:Neoplasm Staging; D010534:Peritoneal Neoplasms / Q000401:mortality / Q000473:pathology / Q000601:surgery / Q000628:therapy*; D010537:Peritoneum / Q000187:drug effects / Q000473:pathology / Q000528:radiation effects / Q000601:surgery; D011446:Prospective Studies; D012189:Retrospective Studies; D016019:Survival Analysis; D016896:Treatment Outcome
chemical_list: D004317:Doxorubicin; D007069:Ifosfamide