J Korean Neurosurg Soc.  1987 Dec;16(4):1201-1210.

Von Hippel-Lindau's Disease: Case Report

• Ryu TH
• Kim SG
• Choe GH
• Yeo HT
• Rhee JK

Abstract

Although cases of von Hippel-Lindau syndrome are not rare, the fact that hemangioblastomans can occur at sites in the nervous system other than the cerebellum is not appreciated. We are reporting a case of VHL complex in 23-year-old male. In this case, there were multiple cerebellar hemangioblastomas, retinal angiomas, multiple spinal hemangioblastomas, syringomyelia, and papillary cystadenoma of epididymis. There was no evidence of polycythe mia or abnormality of other organs. Large cystic cerebellar hemangioblastomas were removed surgically and papillary cystadenoma of epididymis were excised, too. Total laminectomy of T2, T3 and T4 revealed abnormal meningeal varicosities, enlarged spinal cord, inra-axial hemangioblastomas, and syringomyelia.

Keyword

VHL complex; Cerebellar hermangioblastoma; Retinal angioma; Papillary cystadenoma of epididymis; Hemangioblastoma of spinal cord; Syringomyelia

MeSH Terms

Cerebellum
Cystadenoma, Papillary
Epididymis
Hemangioblastoma
Hemangioma
Humans
Laminectomy
Male
Nervous System
Retinaldehyde
Spinal Cord
Syringomyelia
von Hippel-Lindau Disease
Young Adult
Retinaldehyde