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Recurrent desmoplastic small round cell tumor responding to an mTOR inhibitor containing regimen
Author(s) -
Tarek Nidale,
HayesJordan Andrea,
Salvador Laura,
McAleer Mary F.,
Herzog Cynthia E.,
Huh Winston W.
Publication year - 2018
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.26768
Subject(s) - desmoplastic small round cell tumor , medicine , neutropenia , regimen , mucositis , oncology , cyclophosphamide , temsirolimus , discovery and development of mtor inhibitors , surgery , chemotherapy , pi3k/akt/mtor pathway , apoptosis , biochemistry , chemistry
Desmoplastic small round cell tumor (DSRCT) is a rare mesenchymal tumor that typically presents with multiple abdominal masses. Initial treatment is multimodal in nature. Patients with relapsed DSRCT have a poor prognosis, and there are no standard therapies. We report our experience with five patients treated with vinorelbine, cyclophosphamide, and temsirolimus (VCT). Median number of VCT courses delivered was 7 (range 4–14 courses), and partial response was observed in all patients. Median time to progression or relapse was 8.5 months (range 7–16 months). Neutropenia and mucositis were most common toxicities (n = 4 each).