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Annals of Surgical Oncology
Published in: Annals of Surgical Oncology 4/2018

01-04-2018 | Bone and Soft Tissue Sarcomas

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

Authors: Andrea A. Hayes-Jordan, MD, Brian A. Coakley, MD, Holly L. Green, PA-C, LianChun Xiao, MS, Keith F. Fournier, MD, Cynthia E. Herzog, MD, Joseph A. Ludwig, MD, Mary F. McAleer, MD, PhD, Peter M. Anderson, MD, PhD, Winston W. Huh, MD

Published in: Annals of Surgical Oncology | Issue 4/2018

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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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Metadata
Title
Desmoplastic Small Round Cell Tumor Treated with Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy: Results of a Phase 2 Trial
Authors
Andrea A. Hayes-Jordan, MD
Brian A. Coakley, MD
Holly L. Green, PA-C
LianChun Xiao, MS
Keith F. Fournier, MD
Cynthia E. Herzog, MD
Joseph A. Ludwig, MD
Mary F. McAleer, MD, PhD
Peter M. Anderson, MD, PhD
Winston W. Huh, MD
Publication date
01-04-2018
Publisher
Springer International Publishing
Published in
Annals of Surgical Oncology / Issue 4/2018
Print ISSN: 1068-9265
Electronic ISSN: 1534-4681
DOI
https://doi.org/10.1245/s10434-018-6333-9

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