Annals of Surgical Oncology

, Volume 21, Issue 1, pp 220–224 | Cite as

Complete Cytoreduction and HIPEC Improves Survival in Desmoplastic Small Round Cell Tumor

  • Andrea Hayes-Jordan
  • Holly L. Green
  • Heather Lin
  • Pascal Owusu-Agyemang
  • Nancy Fitzgerald
  • Radha Arunkumar
  • Rodrigo Mejia
  • Regina Okhuysen-Cawley
  • Rizalina Mauricio
  • Keith Fournier
  • Joseph Ludwig
  • Peter Anderson
Gastrointestinal Oncology

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.

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Copyright information

© Society of Surgical Oncology 2013

Authors and Affiliations

  • Andrea Hayes-Jordan
    • 1
  • Holly L. Green
    • 2
  • Heather Lin
    • 3
  • Pascal Owusu-Agyemang
    • 4
  • Nancy Fitzgerald
    • 6
  • Radha Arunkumar
    • 4
  • Rodrigo Mejia
    • 2
  • Regina Okhuysen-Cawley
    • 2
  • Rizalina Mauricio
    • 2
  • Keith Fournier
    • 1
  • Joseph Ludwig
    • 5
  • Peter Anderson
    • 2
  1. 1.Department of Surgical OncologyUniversity of Texas, MD Anderson Cancer CenterHoustonUSA
  2. 2.Department of Pediatrics - Patient CareUniversity of Texas, MD Anderson Cancer CenterHoustonUSA
  3. 3.Department of BiostatisticsUniversity of Texas, MD Anderson Cancer CenterHoustonUSA
  4. 4.Department of Anesthesiology & PeriOper MedUniversity of Texas, MD Anderson Cancer CenterHoustonUSA
  5. 5.Department of Sarcoma Medical OncologyUniversity of Texas, MD Anderson Cancer CenterHoustonUSA
  6. 6.Department of Diagnostic RadiologyUniversity of Texas, MD Anderson Cancer CenterHoustonUSA

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