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Pediatr Blood Cancer. 2010 Dec 15;55(7):1431-2. doi: 10.1002/pbc.22755.

Temsirolimus for metastatic desmoplastic small round cell tumor.

Author information

1
Department of Medical Oncology, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands. A.Thijs@aig.umcn.nl

Abstract

Desmoplastic small round cell tumor (DSRCT) is a rare and aggressive neoplasm that mostly occurs in young males. No curative treatment options currently exist for this type of tumor and long-term survival remains poor. In vitro rapamycin induces apoptotic death of JN-DSRCT-1 cells, a possible model for desmoplastic small round cell tumors in which the EWS gene is fused to the WT1 gene. We therefore demonstrate the prolonged activity of temsirolimus, an mTOR-inhibitor, in a patient with DSRCT.

PMID:
20730888
DOI:
10.1002/pbc.22755
[Indexed for MEDLINE]

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