Retinal hemangioblastoma (RH) is the earliest and most common clinical manifestation in Von Hippel-Lindau (VHL) disease. RH can also present in isolation without any evidence of VHL. Clinical course of RH can be stationary or progress to exudation and chronic retinal detachment requiring surgical intervention. We report 3 cases of aggressive RH with extraocular extension in young males causing painful blind eye requiring enucleation. Two of our cases were bilateral involvement and had systemic manifestations of VHL. The third patient had unilateral involvement with no systemic evidence of VHL. This manifestation of RH is rarely reported. Two of our patients with VHL had early manifestations of RH and had undergone multiple cryotherapy sessions as well as retinal detachment surgery for exudative retinal detachment. This differential should be considered in vascular lesion arising from intraocular structures especially in diagnosed patients of VHL. The cases also highlight the aggressive behaviour and long-term progression of RH in some patients despite early treatment.
Ocul Oncol Pathol

中文翻译：

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视网膜血管母细胞瘤眼外延伸三例报告

视网膜血管母细胞瘤 (RH) 是 Von Hippel-Lindau (VHL) 病中最早和最常见的临床表现。RH 也可以在没有任何 VHL 证据的情况下单独存在。RH的临床过程可以是静止的或进展为渗出和需要手术干预的慢性视网膜脱离。我们报告了 3 例侵袭性 RH 与年轻男性的眼外延伸，导致疼痛的失明，需要去核。我们的两个病例是双侧受累，并有 VHL 的全身表现。第三名患者有单侧受累，没有 VHL 的全身证据。这种 RH 的表现很少报道。我们的两名 VHL 患者有 RH 的早期表现，并经历了多次冷冻治疗以及渗出性视网膜脱离的视网膜脱离手术。在眼内结构引起的血管病变中应考虑这种差异，尤其是在确诊的 VHL 患者中。尽管早期治疗，这些病例还突出了一些患者的攻击性行为和 RH 的长期进展。
眼肿瘤病理