Desmoplastic Small Round Cell Tumor (DSRCT) is a rare peritoneal disease affecting children and young adults. Despite a very poor prognosis, long-term survivors have been reported. the aim of the study is to identify in a nation-wide survey patients with a prolonged survival after DSRCT diagnosis and to identify factors associated with a cure.
All consecutive patients treated for DSRCT in 9 French expert centers between 1991 and 2018 were identified and retrospectively analyzed. Patients with a follow-up of less than 2 years were excluded from the analysis. Cure was defined as a disease-free survival of at least 5 years.
100 pts were identified (median age 25, 89% male). 27 had distant metastases at diagnosis. 80 pts underwent upfront chemotherapy and 51 pts were subsequently operated. 20 pts went directly to surgery. Surgery was macroscopically complete (CC0/1) in 50 pts. Intraperitoneal chemotherapy was associated to surgery in 17 pts. 54 pts had postoperative chemotherapy and 26 pts had postoperative whole abdomino-pelvic RT (WAP-RT). After a median follow-up of 124 months (range 23-311), the median overall survival (OS) was 25 months. 1- year, 3-year and 5-year OS rates were 90%, 35% and 4% respectively. 7 patients were considered cured after a median disease-free interval of 100 months (range 22-139). Predictive factor of cure were female sex (HR = 4.46, p = 0.005), median PCI<12 (HR = 4.53, p = 0.005), MD Anderson stage I (HR = 3.97, p = 0.003), CC0/1 (HR = 2.17, p = 0.05) and WAP-RT (HR = 3.41; p = 0.003). Neither Hyperthemic intraperitoneal chemotherapy (HIPEC) nor early postoperative intraperitoneal chemotherapy (EPIC) did increase the rate of cure.
Cure in DSRCT is possible in 7% of patients and is best achieved combining systemic chemotherapy, complete cytoreductive surgery and WAP-RT. Targeted treatments are urgently needed.
Clinical trial identification
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