Uekawa Ken, Nakamura Hideo, Shinojima Naoki, Takezaki Tatsuya, Yano Shigetoshi, Kuratsu Jun-Ichi
Department of Neurosurgery, Kumamoto University School of Medicine, Kumamoto, Kumamoto.
NMC Case Rep J. 2015 Sep 8;3(2):29-33. doi: 10.2176/nmccrj.cr.2015-0012. eCollection 2016 Apr.
Unlike in children, brain stem gliomas in adult are rare and still poorly understood. In addition, most adult brain stem gliomas result predominantly in the pons and are less often found in the medulla oblongata. Here, we report a case of an adult glioma in the medulla oblongata and its molecular biological features. A 46-year-old male presented with gait disturbance, paresthesia, and dysphagia. Magnetic resonance imaging (MRI) showed a diffuse hyper-intensive lesion in the medulla oblongata on a T-weighted image without gadolinium contrast enhancement. We performed an open biopsy and the lesion was pathologically diagnosed as a diffuse astrocytoma. Molecular biological analyses revealed the absence of histone H3.3 mutation (H3F3A K27M), and presence of methylation of O-6-methylguanine-DNA methyltransferase (MGMT) promoter and a mutation in isocitrate dehydrogenase 1 (IDH-1). The patient received local radiotherapy and temozolomide chemotherapy. The patient's symptoms were ameliorated, and MRI showed no tumor growth at 6 months after the initial treatment. Biopsy for brain stem lesions is generally thought to have risk of complications, but if performed minimally, it is useful to diagnose and determine treatment strategy. Obtaining patient characteristics and molecular biological features will provide insight towards therapeutic treatment for adult brain stem gliomas.
与儿童不同,成人脑干胶质瘤较为罕见,目前仍了解甚少。此外,大多数成人脑干胶质瘤主要发生在脑桥,而延髓中较少见。在此,我们报告一例成人延髓胶质瘤病例及其分子生物学特征。一名46岁男性出现步态障碍、感觉异常和吞咽困难。磁共振成像(MRI)显示在T加权图像上延髓有弥漫性高信号病变,无钆对比增强。我们进行了开放性活检,病理诊断该病变为弥漫性星形细胞瘤。分子生物学分析显示不存在组蛋白H3.3突变(H3F3A K27M),存在O-6-甲基鸟嘌呤-DNA甲基转移酶(MGMT)启动子甲基化以及异柠檬酸脱氢酶1(IDH-1)突变。患者接受了局部放疗和替莫唑胺化疗。患者症状得到改善,初始治疗6个月后MRI显示无肿瘤生长。一般认为脑干病变活检有并发症风险,但如果操作微创,对于诊断和确定治疗策略是有用的。获取患者特征和分子生物学特征将为成人脑干胶质瘤的治疗提供思路。