J Korean Neurosurg Soc.  1999 Dec;28(12):1782-1788.

Two Cases of Multiple Hemangioblastomas in a Von Hippel-Lindau Family

• Kwak SW
• Jeun SS
• Lee KS
• Son BC
• Hong YK
• Park CK
• Kim MC
• Kang JK

Abstract

Hemangioblastoma is uncommon, accounting for 1 to 2.5% of all primary neoplasms of the central nervous system. Ten to 20% of hemangioblastomas occur as part of Von Hippel-Lindau disease(VHL). Multiple hemangioblastomas are seen only with VHL and these are seen in up to half of VHL-associated hemangioblastomas. We have treated two cases of multiple hemangioblastomas associated with VHL disease in a family, sister(case I, 48/female) and her brother(case II, 41/male). Both patients had renal and pancreatic cysts in addition to CNS hemangioblastomas. Their hemangioblastomas were removed totally, and then their neurological symptoms had Improved. With a review of the literature, the authors present a family of VHL-associated multiple hemangioblastomas.

Keyword

Multiple hemangioblastomas; Von Hippel-Lindau disease.; Renal cyst; Pancreatic cyst

MeSH Terms

Central Nervous System
Hemangioblastoma*
Humans
Pancreatic Cyst