Xu Shujia, Li Qian, Bian Bingyang, Zhou Hongli, Li Dan
The First Hospital of Jilin University, Changchun, China.
The Second Hospital of Jilin University, Changchun, China.
BMC Ophthalmol. 2020 Nov 3;20(1):437. doi: 10.1186/s12886-020-01706-4.
Hemangioblastomas are rare benign tumours that are most commonly detected in the subtentorium or spinal cord. Optic nerve hemangioblastoma is very rare and is most commonly associated with Von Hippel-Lindau (VHL) syndrome.
Here, we report a case of hemangioblastoma of the optic nerve with bilateral frontal lobe oedema without VHL syndrome, which has not yet been reported. A 51-year-old woman presented with progressive and painless deteriorating vision in the left eye. Magnetic resonance imaging showed a mass at the back of the left orbital optic nerve. Endoscopic-assisted intraorbital tumour resection was performed successfully. The pathological diagnosis was left optic nerve hemangioblastoma.
This is the first reported case of optic nerve hemangioblastoma (HBL) with bilateral frontal lobe oedema.
成血管细胞瘤是一种罕见的良性肿瘤，最常见于小脑幕下或脊髓。视神经成血管细胞瘤非常罕见，最常与冯·希佩尔-林道（VHL）综合征相关。
在此，我们报告一例无VHL综合征的双侧额叶水肿的视神经成血管细胞瘤病例，此前尚未见报道。一名51岁女性因左眼视力进行性无痛性下降就诊。磁共振成像显示左侧眶内视神经后部有一肿块。成功实施了内镜辅助眶内肿瘤切除术。病理诊断为左侧视神经成血管细胞瘤。
这是首例报道的伴有双侧额叶水肿的视神经成血管细胞瘤（HBL）病例。
