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Clinical characteristics and outcomes of pediatric patients with desmoplastic small round cell tumor
- Source :
- Rare Tumors, Vol 8, Iss 1 (2016)
- Publication Year :
- 2016
- Publisher :
- SAGE Publishing, 2016.
-
Abstract
- Desmoplastic small round cell tumor (DSRCT) is a rare malignancy that typically affects pediatric and young adult patients. There are limited data on the clinical features of pediatric DSRCT. We selected patients aged 0-21 years reported to the Surveillance, Epidemiology and End Results Program from 1991-2011. We estimated overall survival using Kaplan-Meier approaches and compared outcomes using the log rank test. The median age of the 95 pediatric patients was 15.3 years (range: 0-21). The majority of tumors originated in the abdomen and pelvis (84.4%) and the majority of patients had distant metastasis (72.6%). A minority of patients received radiation (34%). Overall survival at 5 years was poor (18.1%; 95% confidence interval 10.1-27.9%). Radiation therapy was associated with superior survival. Pediatric patients with DSRCT have significant disease burden. Outcomes for children are poor, though patients selected for radiation appear to have improved survival.
Details
- Language :
- English
- ISSN :
- 20363605 and 20363613
- Volume :
- 8
- Issue :
- 1
- Database :
- Directory of Open Access Journals
- Journal :
- Rare Tumors
- Publication Type :
- Academic Journal
- Accession number :
- edsdoj.b2d6a9aa2c124b7682aa37584ebacb6f
- Document Type :
- article
- Full Text :
- https://doi.org/10.4081/rt.2016.6145