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

  • Gastrointestinal Oncology
  • Published:
Annals of Surgical Oncology Aims and scope Submit manuscript

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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Acknowledgment

The authors thank Judy Roehm for manuscript preparation.

Disclosures

None.

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Authors and Affiliations

  1. Department of Surgical Oncology, University of Texas, MD Anderson Cancer Center, Houston, TX, USA

    Andrea Hayes-Jordan MD & Keith Fournier MD

  2. Department of Pediatrics - Patient Care, University of Texas, MD Anderson Cancer Center, Houston, TX, USA

    Holly L. Green BS, PA-C, Rodrigo Mejia MD, Regina Okhuysen-Cawley MD, Rizalina Mauricio MSN, CCRN, CPNP-AC & Peter Anderson MD

  3. Department of Biostatistics, University of Texas, MD Anderson Cancer Center, Houston, TX, USA

    Heather Lin PhD

  4. Department of Anesthesiology & PeriOper Med, University of Texas, MD Anderson Cancer Center, Houston, TX, USA

    Pascal Owusu-Agyemang MD & Radha Arunkumar MD

  5. Department of Sarcoma Medical Oncology, University of Texas, MD Anderson Cancer Center, Houston, TX, USA

    Joseph Ludwig MD, PhD

  6. Department of Diagnostic Radiology, University of Texas, MD Anderson Cancer Center, Houston, TX, USA

    Nancy Fitzgerald MD

Authors
  1. Andrea Hayes-Jordan MD
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  2. Holly L. Green BS, PA-C
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  3. Heather Lin PhD
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  4. Pascal Owusu-Agyemang MD
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  5. Nancy Fitzgerald MD
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  6. Radha Arunkumar MD
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  7. Rodrigo Mejia MD
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  8. Regina Okhuysen-Cawley MD
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  9. Rizalina Mauricio MSN, CCRN, CPNP-AC
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  10. Keith Fournier MD
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  11. Joseph Ludwig MD, PhD
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  12. Peter Anderson MD
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Corresponding author

Correspondence to Andrea Hayes-Jordan MD.

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Hayes-Jordan, A., Green, H.L., Lin, H. et al. Complete Cytoreduction and HIPEC Improves Survival in Desmoplastic Small Round Cell Tumor. Ann Surg Oncol 21, 220–224 (2014). https://doi.org/10.1245/s10434-013-3269-y

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  • DOI: https://doi.org/10.1245/s10434-013-3269-y

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