Department of Oncology, Akershus University Hospital, Lørenskog, Norway.
Department of Neuropathology, Oslo University Hospital, Oslo, Norway.
Acta Neurochir (Wien). 2019 Feb;161(2):343-349. doi: 10.1007/s00701-019-03800-z. Epub 2019 Jan 17.
Hemangioblastomas (HB) are benign tumors of the central nervous system (CNS) that can appear sporadic or as part of von Hippel-Lindau (VHL) disease. It is often curable with surgical resection, but upon relapse, the disease exhibits a treatment-refractory course.
A patient treated for sporadic cerebellar HB relapsed 12 years post-surgery. She developed disseminated disease throughout the CNS, including leptomeningeal manifestations. Repeat surgery and craniospinal radiation therapy were unsuccessful.
This case is in line with previous publications on disseminated non-VHL HB. Available treatment options are inefficient, emphasizing the need for improved understanding of HB biology to identify therapeutic targets.
血管母细胞瘤（HB）是一种中枢神经系统（CNS）的良性肿瘤，可表现为散发性或作为 von Hippel-Lindau（VHL）病的一部分。通过手术切除通常可以治愈，但复发后，疾病表现出难治性病程。
一名接受散发性小脑 HB 治疗的患者在手术后 12 年复发。她的疾病在中枢神经系统中广泛扩散，包括软脑膜表现。重复手术和全脑脊髓放射治疗均不成功。
该病例与先前关于弥散性非 VHL HB 的出版物一致。现有的治疗选择效率低下，这强调了需要更好地了解 HB 生物学以确定治疗靶点。
