Familial Occurrence of Von hippel-Lindau Disease: Case Report.
- Author:
Hang Woo LEE
1
;
Sun Il LEE
;
Yong Tae JUNG
;
Byung Ook CHOI
;
Soo Chun KIM
;
Jae Hong SIM
Author Information
1. Department of Neurosurgery, College of Medicine, Inje University, Paik Hospital, Pusan, Korea.
- Publication Type:Original Article
- Keywords:
Hemangioblastoma;
Von Hippel-Lindau disease;
Familial occurrence;
Autosomal dominant
- MeSH:
Brain Neoplasms;
Central Nervous System;
Hemangioblastoma;
Hemangioma;
Humans;
Kidney;
Male;
Pancreas;
Pheochromocytoma;
Retina;
von Hippel-Lindau Disease*
- From:Journal of Korean Neurosurgical Society
1996;25(7):1500-1508
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Hemangioblastoma is an uncommon tumor of the central nervous system, accounting for only 1 % to 2.5% of all intracranial neoplasms. Hemangioblastoma can occur either sporadically or as a manifestation of von Hippel-Lindau diseasean inherited disorder of the autosomal dominant trait-characterized by tumors or tumor-like lesions developing in several organs including angioma of retina, pheochromocytoma, cyst or carcinoma in kidney and pancreas. We have encountered a family in which two male members were histologically diagnosed as suffering from von Hippel-Lindau disease and another male member was clinically suspected of suffering from spinal hemangioblastoma. The pathophysiology and genetic aspect of von Hippel-Lindau disease are discussed with review of literatures.
