title: Abdominal desmoplastic small round cell tumor: multimodal treatment combining chemotherapy, surgery, and radiotherapy is the best option.
abstract: BACKGROUND
  With nearly 450 cases reported since 1991, desmoplastic small round cell tumor (DSRCT) is a rare abdominal tumor typically arising in adolescent and young adult white men. With no large series described, the best therapeutic strategy remains unclear.
  
  
  METHODS
  All consecutive patients treated in our tertiary care center between January 1991 and December 2013 for a DSRCT were retrospectively studied.
  
  
  RESULTS
  Thirty-eight patients with a median age of 27 years (range 13-57 years) were identified; 71 % were men. At the time of diagnosis, 47.4 % patients had extraperitoneal metastases (EPM): 78 % were located in the liver and 11 % were located in the lungs. Fourteen patients (37 %) were treated exclusively with systemic chemotherapy, with a median survival of 21.1 months. Twenty-three patients underwent surgery, 12 (52 %) experienced complete removal of all macroscopic disease, 5 (21.7 %) received additional intraperitoneal chemotherapy, and 7 (30 %) received postoperative whole abdominopelvic radiotherapy (WAP RT). With a median follow-up of 59.9 months, the median survival was 37.7 months, and the median disease-free survival was 15.5 months. The factors predictive of 3-year overall survival were the absence of EPM, complete surgical resection, postoperative WAP RT, and postoperative chemotherapy. The intraperitoneal chemotherapy had no impact on overall survival.
  
  
  CONCLUSIONS
  DSRCT is a rare and aggressive disease. In patients without EPM, a multimodal treatment combining systemic chemotherapy, complete macroscopic resection, and postoperative WAP RT could enable prolonged survival. No benefit of surgery was demonstrated for patients with EPM. The value of associated hyperthermic intraperitoneal chemotherapy remains unproven.
authors.affiliation: Department of Surgical Oncology, Gustave Roussy Cancer Center, Villejuif, France.
authors.forename: Charles
authors.initials: C
authors.lastname: Honoré
authors.forename: Koceila
authors.initials: K
authors.lastname: Amroun
authors.forename: Laurence
authors.initials: L
authors.lastname: Vilcot
authors.forename: Olivier
authors.initials: O
authors.lastname: Mir
authors.forename: Julien
authors.initials: J
authors.lastname: Domont
authors.forename: Philippe
authors.initials: P
authors.lastname: Terrier
authors.forename: Axel
authors.initials: A
authors.lastname: Le Cesne
authors.forename: Cecile
authors.initials: C
authors.lastname: Le Péchoux
authors.forename: Sylvie
authors.initials: S
authors.lastname: Bonvalot
mesh_terms: D000008:Abdominal Neoplasms / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D000293:Adolescent; D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D003131:Combined Modality Therapy; D058405:Desmoplastic Small Round Cell Tumor / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007036:Hypothermia, Induced; D008113:Liver Neoplasms / Q000401:mortality / Q000556:secondary / Q000628:therapy*; D008175:Lung Neoplasms / Q000401:mortality / Q000556:secondary / Q000628:therapy*; D008207:Lymphatic Metastasis; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D009367:Neoplasm Staging; D010534:Peritoneal Neoplasms / Q000401:mortality / Q000556:secondary / Q000628:therapy*; D011379:Prognosis; D012189:Retrospective Studies; D015996:Survival Rate; D062606:Tertiary Care Centers; D055815:Young Adult