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Desmoplastic Small Round Cell Tumor Treated with Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy: Results of a Phase 2 Trial

  • Bone and Soft Tissue Sarcomas
  • Published:
Annals of Surgical Oncology Aims and scope Submit manuscript

Abstract

Background

Desmoplastic small round cell tumor (DSRCT) is a rare sarcoma that primarily affects adolescents and young adults. Patients can present with many peritoneal implants. We conducted a phase 2 clinical trial utilizing cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS-HIPEC) with cisplatin for DSRCT and pediatric-type abdominal sarcomas.

Patients and Methods

A prospective cohort study was performed on 20 patients, who underwent CRS-HIPEC procedures, with cisplatin from 2012 to 2013. All patients were enrolled in the phase 2 clinical trial. Patients with extraabdominal disease and in whom complete cytoreduction (CCR0–1) could not be achieved were excluded. All outcomes were recorded.

Results

Fourteen patients had DSRCT, while five patients had other sarcomas. One patient had repeat HIPEC. Patients with DSRCT had significantly longer median overall survival after surgery than patients with other tumors (44.3 vs. 12.5 months, p = 0.0013). The 3-year overall survival from time of diagnosis for DSRCT patients was 79 %. Estimated median recurrence-free survival (RFS) was 14.0 months. However, RFS for patients with DSRCT was significantly longer than for non-DSRCT patients (14.9 vs. 4.5 months, p = 0.0012). Among DSRCT patients, those without hepatic or portal metastases had longer median RFS than those with tumors at these sites (37.9 vs. 14.3 months, p = 0.02). In 100 % of patients without hepatic or portal metastasis, there was no peritoneal disease recurrence after CRS-HIPEC.

Conclusions

Complete CRS-HIPEC with cisplatin is effective in select DSRCT patients. DSRCT patients with hepatic or portal metastasis have poorer outcomes.

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Acknowledgements

Supported in part by MD Anderson’s Cancer Center Support (Core) Grant, NIH/NCI P30 CA016672, and by Young Texans Against Cancer (YTAC).

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Authors and Affiliations

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

    Andrea A. Hayes-Jordan MD, Brian A. Coakley MD & Keith F. Fournier MD

  2. Department of Pediatric Surgery, Children’s Hospital of Los Angeles, Los Angeles, CA, USA

    Holly L. Green PA-C

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

    LianChun Xiao MS

  4. Division of Pediatrics, The University of Texas MD Anderson Cancer Center, Houston, TX, USA

    Cynthia E. Herzog MD & Winston W. Huh MD

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

    Joseph A. Ludwig MD

  6. Department of Radiation Oncology, The University of Texas MD Anderson Cancer Center, Houston, USA

    Mary F. McAleer MD, PhD

  7. Department of Pediatric Hematology Oncology and Blood and Marrow Transplantation, Cleveland Clinic, Cleveland, OH, USA

    Peter M. Anderson MD, PhD

Authors
  1. Andrea A. Hayes-Jordan MD
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  2. Brian A. Coakley MD
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  3. Holly L. Green PA-C
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  4. LianChun Xiao MS
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  5. Keith F. Fournier MD
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  6. Cynthia E. Herzog MD
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  7. Joseph A. Ludwig MD
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  8. Mary F. McAleer MD, PhD
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  9. Peter M. Anderson MD, PhD
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  10. Winston W. Huh MD
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Corresponding author

Correspondence to Andrea A. Hayes-Jordan MD.

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Hayes-Jordan, A.A., Coakley, B.A., Green, H.L. et al. Desmoplastic Small Round Cell Tumor Treated with Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy: Results of a Phase 2 Trial. Ann Surg Oncol 25, 872–877 (2018). https://doi.org/10.1245/s10434-018-6333-9

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  • DOI: https://doi.org/10.1245/s10434-018-6333-9

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