Weaver Kristin J, Crawford Lexi M, Bennett Jeffrey A, Rivera-Zengotita Marie L, Pincus David W
Departments of 1 Neurosurgery.
Radiology, and.
J Neurosurg Pediatr. 2017 Oct;20(4):347-351. doi: 10.3171/2017.5.PEDS16402. Epub 2017 Jul 28.
Angiocentric glioma is a rare tumor that was recognized by the WHO Classification of Tumours of the Central Nervous System as a distinct clinicopathological entity in 2007. Since this initial description, the vast majority of cases of angiocentric glioma reported in the literature have involved tumors of the cerebral hemispheres. To date, only 1 case of angiocentric glioma arising from the posterior midbrain has been reported. The authors present the cases of 2 pediatric patients who were found to have brainstem angiocentric gliomas. The clinical course, radiological and pathological features, treatment, and follow-up are described. The first case is one of a 5-year-old girl who presented with double vision, headache, and nausea and was found to have a midbrain lesion with pathological features consistent with angiocentric glioma. She was treated with resection and endoscopic third ventriculostomy (ETV), followed by close observation and serial neuroimaging. The second case is one of a 6-year-old boy who presented with progressive mouth drooping and problems with balance. He was found to have a pontine lesion with pathological features consistent with angiocentric glioma. This patient was treated with ETV, followed by close observation and serial neuroimaging. This report includes 6 and 1.5 years of follow-up of the patients, respectively. While there are limited data regarding the prognosis or long-term management of patients with brainstem angiocentric gliomas, the cases described in this report suggest an indolent course for this tumor, similar to the course of angiocentric gliomas located in the cerebral hemispheres.
血管中心性胶质瘤是一种罕见肿瘤，2007年被世界卫生组织中枢神经系统肿瘤分类确认为一种独特的临床病理实体。自最初描述以来，文献报道的绝大多数血管中心性胶质瘤病例都涉及大脑半球肿瘤。迄今为止，仅报告过1例起源于中脑后部的血管中心性胶质瘤。作者介绍了2例被发现患有脑干血管中心性胶质瘤的儿科患者的病例。描述了其临床病程、影像学和病理特征、治疗及随访情况。第一例是一名5岁女孩，出现复视、头痛和恶心症状，被发现中脑有病变，病理特征符合血管中心性胶质瘤。她接受了手术切除和内镜下第三脑室造瘘术（ETV），随后密切观察并进行系列神经影像学检查。第二例是一名6岁男孩，出现进行性口角下垂和平衡问题。他被发现脑桥有病变，病理特征符合血管中心性胶质瘤。该患者接受了ETV治疗，随后密切观察并进行系列神经影像学检查。本报告分别对这两名患者进行了6年和1.5年的随访。虽然关于脑干血管中心性胶质瘤患者的预后或长期管理的数据有限，但本报告中描述的病例表明该肿瘤病程进展缓慢，类似于位于大脑半球的血管中心性胶质瘤的病程。
