| Literature DB >> 28784858 |
Anastasia Drobysheva1,2, Laura J Klesse3,4, Daniel C Bowers3,4, Veena Rajaram1,2, Dinesh Rakheja1,2,3, Charles F Timmons1,2, Jason Wang1,2, Korgun Koral5,6, Lynn Gargan4, Erica Ramos2, Jason Y Park1,2,7.
Abstract
This report presents a series of 5 pediatric patients with disseminated pilocytic astrocytomas and frequent nonfusion activating mutations. Genetic variants in these patients' tumors include BRAF p.Val600Glu, BRAF p.Val600Asp, and KRAS p.Gly60_Gln62ins7. The 2 patients with BRAF-mutated tumors were treated with dabrafenib or a combination of dabrafenib plus trametinib. The patients had either near complete resolution of the primary tumor (BRAF p.Val600Glu) or a stable primary tumor (BRAF p.Val600Asp). Both patients showed improvement in leptomeningeal dissemination without significant toxicity. Genomic testing of disseminated pilocytic astrocytomas, particularly those arising at extracerebellar locations, may result in the identification of mutations associated with ERK/MAPK activation. Patients with these activating mutations may benefit from targeted therapies.Entities:
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Year: 2017 PMID： 28784858 DOI： 10.6004/jnccn.2017.0139
Source DB: PubMed Journal: J Natl Compr Canc Netw ISSN： 1540-1405 Impact factor: 11.908