Department of Neurosurgery, National Defense Medical College, Saitama, Japan.
Acta Neurol Belg. 2011 Dec;111(4):353-6.
A 33-year-old male presented with a headache. He had a history of a previous surgical procedure for excisions of cervical spine hemangioblastomas 13 years prior He had a family history of von-Hippel Lindau (VHL) disease, and a VHL mutation was identified. Brain magnetic resonance imaging showed enhanced mass lesions in both the third and right lateral ventricles as well as in the cerebellum. The lesion in the right lateral ventricle also had a cystic component. Two-staged resections of the tumors in the third and right lateral ventricles and ventriculo-peritoneal shunt were performed. A histopathological specimen was compatible with a hemangioblastoma. Supratentorial intraventricular hemangioblastomas are extremely rare. We reviewed the literature and discussed the features.
一位 33 岁男性因头痛就诊。他在 13 年前曾因颈椎血管瘤切除术接受过一次手术。他有 von-Hippel Lindau(VHL)病家族史,且发现 VHL 基因突变。脑部磁共振成像显示双侧第三脑室和右侧侧脑室以及小脑有强化的肿块病变。右侧侧脑室的病变还有一个囊状部分。对第三脑室和右侧侧脑室的肿瘤进行了两阶段切除和脑室-腹腔分流术。组织病理学标本与血管母细胞瘤一致。幕上脑室内血管母细胞瘤极为罕见。我们回顾了文献并讨论了其特征。