Burdenko Neurosurgical Center, Moscow, Russia.
Zh Vopr Neirokhir Im N N Burdenko. 2023;87(5):78-84. doi: 10.17116/neiro20238705178.
Hemangioblastoma is a benign tumor of the central nervous system arising sporadically or as a component of Von Hippel-Lindau disease. Von Hippel-Lindau disease is a rare autosomal dominant hereditary syndrome with various phenotypes caused by VHL gene variants. To date, only about 40 cases of optic nerve hemangioblastoma have been described in the literature. Stereotactic irradiation may be effective for supratentorial hemangioblastomas including lesions of optic nerves. The authors describe a rare case of stereotactic irradiation of intraorbital hemangioblastoma of the optic nerve in a patient with Von Hippel-Lindau disease.
血管母细胞瘤是一种中枢神经系统良性肿瘤，可散发发生，也可作为 Von Hippel-Lindau 病的一部分。Von Hippel-Lindau 病是一种罕见的常染色体显性遗传性综合征，由 VHL 基因突变引起多种表型。迄今为止，文献中仅描述了约 40 例视神经血管母细胞瘤。立体定向放疗可能对包括视神经病变在内的幕上血管母细胞瘤有效。作者描述了一例 Von Hippel-Lindau 病患者的视神经眶内血管母细胞瘤的立体定向放疗的罕见病例。
