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Temsirolimus for metastatic desmoplastic small round cell tumor
Author(s) -
Thijs Anna M.J.,
van der Graaf Winette T.A.,
van Herpen Carla M.L.
Publication year - 2010
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.22755
Subject(s) - temsirolimus , desmoplastic small round cell tumor , discovery and development of mtor inhibitors , medicine , cancer research , blood cancer , cancer , neoplasm , pi3k/akt/mtor pathway , pathology , oncology , apoptosis , immunohistochemistry , biology , genetics
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. Pediatr Blood Cancer. 2010;55:1431–1432. © 2010 Wiley‐Liss, Inc.