Two Cases of Multiple Hemangioblastomas in a Von Hippel-Lindau Family.
- Author:
Seung Won KWAK
1
;
Sin Soo JEUN
;
Kwan Sung LEE
;
Byoung Cheol SON
;
Yong Kil HONG
;
Chun Keun PARK
;
Moon Chan KIM
;
Joon Ki KANG
Author Information
1. Department of Neurosurgery, Catholic University Medical College, Seoul, Korea.
- Publication Type:Case Report
- Keywords:
Multiple hemangioblastomas;
Von Hippel-Lindau disease.;
Renal cyst;
Pancreatic cyst
- MeSH:
Central Nervous System;
Hemangioblastoma*;
Humans;
Pancreatic Cyst
- From:Journal of Korean Neurosurgical Society
1999;28(12):1782-1788
- CountryRepublic of Korea
- Language:Korean
-
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.
