Familial Occurrence of Von hippel-Lindau Disease: Case Report
Journal of Korean Neurosurgical Society
;
: 1500-1508, 1996.
Article
in Korean
| WPRIM
| ID: wpr-18332
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.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Pancreas
/
Pheochromocytoma
/
Retina
/
Brain Neoplasms
/
Central Nervous System
/
Hemangioblastoma
/
Hemangioma
/
Von Hippel-Lindau Disease
/
Kidney
Limits:
Humans
/
Male
Language:
Korean
Journal:
Journal of Korean Neurosurgical Society
Year:
1996
Type:
Article
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