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Desmoplastic Small Round Cell Tumor: Long-Term Complications After Cytoreduction and Hyperthermic Intraperitoneal Chemotherapy

  • Peritoneal Surface Malignancy
  • Published:
Annals of Surgical Oncology Aims and scope Submit manuscript

Abstract

Background

Desmoplastic small round cell tumor (DSRCT) is a rare intra-abdominal soft tissue sarcoma affecting adolescents and young adults. Cytoreduction, hyperthermic intraperitoneal chemotherapy (CRS/HIPEC), and adjuvant radiotherapy may improve local control. We review our experience with patients who undergo CRS/HIPEC and adjuvant radiotherapy for DSRCT.

Methods

A retrospective review was performed for patients with DSRCT from 2013 to 2017 who underwent CRS/HIPEC. Clinicopathologic, operative, and outcome data were reviewed.

Results

Ten CRS/HIPEC procedures were performed for nine patients (7 males, 6 Caucasian, median age 19 years (range 10–24)). Four patients presented with extra-abdominal disease; five had liver involvement. The median peritoneal cancer index was 16 (range 5–20). All received neoadjuvant chemotherapy. CCR 0/1 resection was possible in nine patients. Major complications occurred in four with no operative mortalities. All received adjuvant chemotherapy, seven received radiation therapy, and three received stem-cell transplant. All but one patient recurred after treatment. The median recurrence-free and overall survival (OS) were 12 and 45 months (95% confidence interval 35.1–54.9) respectively, with a 3-year OS of 55%. Long-term parenteral nutrition was required in eight for a median of 261 days (range 37–997). Clinically significant long-term complications requiring further surgery included gastroparesis (N = 1), small bowel obstruction (N = 3) and hemorrhagic cystitis (N = 2).

Conclusions

Multimodal therapy for DSRCT consisting of multiagent neoadjuvant chemotherapy, CRS/HIPEC, adjuvant chemotherapy, and radiation therapy is associated with potential cumulative toxicity. Recurrence after resection is common. Prolonged parenteral nutrition may be necessary, and late gastrointestinal and genitourinary complications may require additional treatment.

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Acknowledgment

The authors thank Lynn Wynn, Amy Kimble, and Dina Darby for the care of all patients treated in this series.

Funding

This work was supported in part by the R25CA23944 Grant from the National Cancer Institute.

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Authors and Affiliations

  1. Division of Surgical Oncology, Department of Surgery, University of Tennessee Health Science Center, Memphis, TN, 38163, USA

    Zachary E. Stiles DO, Andrew J. Murphy MD, Andrew M. Davidoff MD, Paxton V. Dickson MD, Evan S. Glazer MD & Jeremiah L. Deneve DO

  2. Department of Surgery, St Jude Children’s Research Hospital, Memphis, TN, USA

    Andrew J. Murphy MD & Andrew M. Davidoff MD

  3. Division of Anesthesiology, Department of Pediatric Medicine, St Jude Children’s Research Hospital, Memphis, TN, USA

    Doralina L. Anghelescu MD & Christina-Lin Brown BS

  4. Department of Medical Oncology, St Jude Children’s Research Hospital, Memphis, TN, USA

    Michael W. Bishop MD, MS, Wayne L. Furman MD & Alberto S. Pappo MD

  5. Department of Radiation Oncology, St Jude Children’s Research Hospital, Memphis, TN, USA

    John T. Lucas Jr. MS, MD

Authors
  1. Zachary E. Stiles DO
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  2. Andrew J. Murphy MD
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  3. Doralina L. Anghelescu MD
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  4. Christina-Lin Brown BS
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  5. Andrew M. Davidoff MD
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  6. Paxton V. Dickson MD
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  7. Evan S. Glazer MD
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  8. Michael W. Bishop MD, MS
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  9. Wayne L. Furman MD
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  11. John T. Lucas Jr. MS, MD
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  12. Jeremiah L. Deneve DO
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Correspondence to Jeremiah L. Deneve DO.

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Stiles, Z.E., Murphy, A.J., Anghelescu, D.L. et al. Desmoplastic Small Round Cell Tumor: Long-Term Complications After Cytoreduction and Hyperthermic Intraperitoneal Chemotherapy. Ann Surg Oncol 27, 171–178 (2020). https://doi.org/10.1245/s10434-019-07339-2

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  • DOI: https://doi.org/10.1245/s10434-019-07339-2

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