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林岛氏病(Von Hippel-indau’s Disease,VHL)是一种常染色体显性遗传病。它包括一系列新生物,最常见有视网膜血管瘤、小脑血管母细胞瘤,肾囊肿、肾癌和嗜咯细胞瘤;较少见的病症还包括毛细血管痣,胰腺囊肿、副睾囊肿、红细胞增多症和脊髓空洞症。VHL病的诊断依Melmon和Rosen(1964)提出的标准:在中枢神经系统(CNS)内有两个以上的血管母细胞瘤,或CNS内有一个孤立的血管母细胞瘤并伴有该病的内脏病变以及有家族史表现该病的任一病症者均可确诊。
Von Hippel-indau’s Disease (VHL) is an autosomal dominant disease. It includes a range of neoplasms, most commonly retinal hemangiomas, cerebellar hemangioblastomas, renal cysts, renal carcinomas and pyorocytoma. Less common disorders include capillary nevus, pancreatic cysts, epidural cysts, erythrocytes Increased disease and syringomyelia. Diagnosis of VHL disease According to the criteria proposed by Melmon and Rosen (1964), there are two or more hemangioblastomas in the central nervous system (CNS) or an isolated hemangioblastoma in the CNS accompanied by the disease Visceral and family history of the disease as well as any of the symptoms can be diagnosed.