Abstract
BackgroundDesmoplastic Small Round Cell Tumor (DSRCT) is a rare disease affecting predominantly children and young adults and for which the benefit of hyperthermic intraperitoneal chemotherapy (HIPEC) after complete cytoreductive surgery (CCRS) remains unknown.MethodsTo identify patients with DSRCT without extraperitoneal metastases (EPM) who underwent CCRS between 1991 and 2015, a retrospective nation-wide survey was conducted by crossing the prospective and retrospective databases of the French Network for Rare Peritoneal Malignancies, French Reference Network in Sarcoma Pathology, French Sarcoma Clinical Network and French Pediatric Cancer Society.ResultsAmong the 107 patients with DSRCT, 48 had no EPM and underwent CCRS. The median peritoneal cancer index (PCI) was 9 (range: 2–27). Among these 48 patients, 38 (79%) had pre- and/or postoperative chemotherapy and 23 (48%) postoperative whole abdominopelvic radiotherapy (WAP-RT). Intraperitoneal chemotherapy was administered to 11 patients (23%): two received early postoperative intraperitoneal chemotherapy (EPIC) and nine HIPEC. After a median follow-up of 30 months, the median overall survival (OS) of the entire cohort was 42 months. The 2-y and 5-y OS were 72% and 19%. The 2-y and 5-y disease-free survival (DFS) were 30% and 12%. WAP-RT was the only variable associated with longer peritoneal recurrence-free survival and DFS after CCRS. The influence of HIPEC/EPIC on OS and DFS was not statistically conclusive.ConclusionThe benefit of HIPEC is still unknown and should be evaluated in a prospective trial. The value of postoperative WAP-RT seems to be confirmed.
Highlights
Desmoplastic Small Round Cell Tumor (DSRCT) is a rare abdominal disease affecting predominantly children and young adults [1,2,3]
Intraperitoneal chemotherapy was administered to 11 patients (23%): two received early postoperative intraperitoneal chemotherapy (EPIC) and nine hyperthermic intraperitoneal chemotherapy (HIPEC)
whole abdominopelvic radiotherapy (WAP-RT) was the only variable associated with longer peritoneal recurrence-free survival and diseasefree survival (DFS) after complete cytoreductive surgery (CCRS)
Summary
Desmoplastic Small Round Cell Tumor (DSRCT) is a rare abdominal disease affecting predominantly children and young adults [1,2,3]. No benefit of surgery was found in patients with EPM [9]. In the absence of comparative studies, there is no consensus concerning the optimal locoregional treatment and the value of hyperthemic intraperitoneal chemotherapy (HIPEC) is not determined. The aim of the present study was to analyze nation-wide in France, the treatment of DSRCT without EPM to determine the potential benefit of HIPEC after macroscopically complete surgery. Desmoplastic Small Round Cell Tumor (DSRCT) is a rare disease affecting predominantly children and young adults and for which the benefit of hyperthermic intraperitoneal chemotherapy (HIPEC) after complete cytoreductive surgery (CCRS) remains unknown
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