title: Complete cytoreduction and HIPEC improves survival in desmoplastic small round cell tumor.
abstract: BACKGROUND
  Desmoplastic small round cell tumor (DSRCT) is a rare tumor of adolescents and young adults. Less than 100 cases per year are reported in North America. Extensive peritoneal metastases are characteristic of this disease. We performed cytoreductive surgery and hyperthermic peritoneal perfusion with chemotherapy (HIPEC) using cisplatin (CDDP) for DSRCT.
  
  
  METHODS
  A retrospective cohort study was performed on 26 pediatric and adult patients who underwent cytoreduction/HIPEC using CDDP for DSRCT at a single cancer center. Neoadjuvant chemotherapy, adjuvant chemotherapy, and postoperative enteral nutrition were given to all patients. Postoperative radiation therapy was given to most patients. Follow-up was from 6 months to 6 years. Outcome variables were evaluated for disease-free and overall survival (OS).
  
  
  RESULTS
  Five patients (19 %) were less than 12 years of age at surgery. Patients who had disease outside the abdomen at surgery had a larger risk of recurrence or death than those who did not (p = 0.0158, p = 0.0393 time from surgery to death respectively). Age, liver metastasis, and peritoneal cancer index level did not significantly predict disease-free or OS. Patients who had CR0 or CR1 and HIPEC had significantly longer median survival compared with patients who had HIPEC and CR2 cytoreduction (63.4 vs. 26.7 months).
  
  
  CONCLUSIONS
  HIPEC may be an effective therapy for children and young adults with DSRCT. Patients with DSRCT require complete cytoreduction before HIPEC to optimize outcome. Patients with DSRCT and disease outside the abdomen at the time of surgery do not benefit from HIPEC.
authors.affiliation: Department of Surgical Oncology, University of Texas, MD Anderson Cancer Center, Houston, TX, USA, ahjordan@mdanderson.org.
authors.forename: Andrea
authors.initials: A
authors.lastname: Hayes-Jordan
authors.forename: Holly L
authors.initials: HL
authors.lastname: Green
authors.forename: Heather
authors.initials: H
authors.lastname: Lin
authors.forename: Pascal
authors.initials: P
authors.lastname: Owusu-Agyemang
authors.forename: Nancy
authors.initials: N
authors.lastname: Fitzgerald
authors.forename: Radha
authors.initials: R
authors.lastname: Arunkumar
authors.forename: Rodrigo
authors.initials: R
authors.lastname: Mejia
authors.forename: Regina
authors.initials: R
authors.lastname: Okhuysen-Cawley
authors.forename: Rizalina
authors.initials: R
authors.lastname: Mauricio
authors.forename: Keith
authors.initials: K
authors.lastname: Fournier
authors.forename: Joseph
authors.initials: J
authors.lastname: Ludwig
authors.forename: Peter
authors.initials: P
authors.lastname: Anderson
mesh_terms: D000293:Adolescent; D000328:Adult; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D017024:Chemotherapy, Adjuvant; D010478:Chemotherapy, Cancer, Regional Perfusion; D002648:Child; D002675:Child, Preschool; D002945:Cisplatin / Q000627:therapeutic use*; D003131:Combined Modality Therapy; D058405:Desmoplastic Small Round Cell Tumor / Q000401:mortality* / Q000473:pathology / Q000601:surgery / Q000628:therapy; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006979:Hyperthermia, Induced; D008113:Liver Neoplasms / Q000401:mortality* / Q000556:secondary / Q000601:surgery / Q000628:therapy; D008207:Lymphatic Metastasis; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local / Q000401:mortality* / Q000473:pathology / Q000628:therapy; D009367:Neoplasm Staging; D011379:Prognosis; D012189:Retrospective Studies; D015996:Survival Rate; D055815:Young Adult
chemical_list: D000970:Antineoplastic Agents; D002945:Cisplatin