Vemurafenib Treatment of BRAF V600E-Mutated Malignant Peripheral Nerve Sheath Tumor

被引：17

作者：

Kaplan, Henry G. ^{[1
]}

机构：

[1] Swedish Canc Inst, Seattle, WA 98104 USA

来源：

JOURNAL OF THE NATIONAL COMPREHENSIVE CANCER NETWORK | 2013年 / 11卷 / 12期

关键词：

MEK INHIBITION; RESISTANCE; MUTATIONS; EFFICACY; MELANOMA; TARGET; V600E; RAF;

D O I：

10.6004/jnccn.2013.0173

中图分类号：

R73 [肿瘤学];

学科分类号：

100214 ;

摘要：

No effective systemic treatment exists for malignant peripheral nerve sheath tumors (MPNSTs). These tumors have been reported to show increased activity in the mitogen-activated protein kinase pathway from the loss of neurofibromatosis-1 regulation and occasionally from BRAF V600E mutation. A patient with sporadic metastatic MPNST and the BRAF V600E mutation was treated with standard doses of sorafenib and later vemurafenib and followed for response. The patient showed a rapid but modest and transient response to sorafenib and a very dramatic response to vemurafenib. This case represents the first report of successful systemic treatment of MPNST with an inhibitor of the BRAF V600E mutation. It will be important to define the general utility of this approach and related therapies in this disease.

引用

页码：1466 / 1470

页数：5

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