1454P - Desmoplastic small round cell tumor (DSRCT) in adult population: Retrospective analysis

Date 29 September 2014
Event ESMO 2014
Session Poster Display session
Topics Soft Tissue Sarcomas
Presenter Tatiana Correa
Citation Annals of Oncology (2014) 25 (suppl_4): iv494-iv510. 10.1093/annonc/mdu354
Authors T.S. Correa1, A.M.G. Andrade2, D.A. Toloi1, C. Venchiarutti2, V.P. Camargo1, M.S. Goncalves1, P.M. Hoff1
  • 1Oncologia Clínica, 5º Andar, Instituto do Cancer do Estado de São Paulo, 01246000 - Sao Paulo/BR
  • 2Oncologia Clinica, Instituto do Cancer do Estado de Sao Paulo, Sao Paulo/BR



DSRCT is a rare and aggressive cancer that affects children and young adults with a predominance of males. There is no standard treatment, and the prognosis is poor. In this retrospective study, we analyzed epidemiologic data, treatment and the overall survival of patients (pts) treated at two Brazilian oncology centers: Instituto do Câncer do Estado de São Paulo and Hospital Sirio-Libanês.


Retrospective analysis of pts with DSRCT treated between January 2007 and November 2012. Overall survival was estimated by the Kaplan-Meier method. The probability of a type I error was specified at 0.05 with use of a stratified log-rank test.


In this period, 11 pts were evaluated. The median age was 25 years, most pts were male (81%), ECOG were 0-1 in 8 and 2 in 3 pts. The primary tumor was mostly in the peritoneum (82%), and other sites were mediastinum and uterine cervix. The initial disease was limited to the peritoneum in 18%. The more frequent metastatic sites were liver (n=5), pleura (n=5), lung (n=3) and lymph nodes (n=3). Most pts received chemotherapy (10 in 11) and 7 were exposed to the regimen VAC (vincristine, doxorubicin and cyclophosphamide) in first line. Other regimens were platinum doublets and ifosfamide plus etoposide. The chemotherapy regimen used was not predictive of improved survival. The time free of progression for the first line was 19.4 weeks. Pts had a median of 3 lines of treatment, and some received radiation therapy (n=3). Survival from diagnosis was 113 weeks and the median overall survival from beginning of treatment was 108 weeks, with a favorable trend for pts with better performance status (ECOG 0/1=180 weeks; 2= 70 weeks p=0,134). Cytoreduction surgery was performed in 5 patients, with no statistical difference in survival (85, 4 vs. 70 weeks; p=0,554).


This is the largest series of Brazilian DSRCT reported. We confirmed that this is a highly aggressive neoplasia. Despite the lack of a standard treatment, our study suggests that surgical treatment and multiagent chemotherapy might yield better disease control, with a propensity to better survival.


All authors have declared no conflicts of interest.