Hussain Faiz, Horbinski Craig M, Chmura Steven J, Yamini Bakhtiar, Lukas Rimas V
Jackson Park Hospital.
Department of Pathology, Northwestern University.
Neurologist. 2018 Sep;23(5):163-166. doi: 10.1097/NRL.0000000000000194.
Pleomorphic xanthoastrocytoma (PXA) is a rare primary central nervous system tumor which frequently harbors mutations in BRAF. Anaplastic PXA follow a more aggressive course than their nonanaplastic counterparts. We present the case of an anaplastic PXA initially treated with the BRAF inhibitor vemurafenib. After progression of disease the MEK inhibitor trametinib was added to the regimen leading to radiographic improvement. The rationale for combined BRAF and MEK inhibition in PXA is reviewed.
多形性黄色瘤型星形细胞瘤（PXA）是一种罕见的原发性中枢神经系统肿瘤，常伴有BRAF基因突变。间变性PXA的病程比非间变性PXA更具侵袭性。我们报告了一例间变性PXA患者，最初接受BRAF抑制剂维莫非尼治疗。疾病进展后，在治疗方案中加入了MEK抑制剂曲美替尼，从而使影像学表现得到改善。本文对PXA联合抑制BRAF和MEK的理论依据进行了综述。
