Korean J Urol.  2005 Jan;46(1):93-95.

Cystic Renal Cell Carcinoma Associated with Von Hippel-Lindau Disease Treated by Nephron Sparing Surgery

Affiliations
  • 1Department of Urology, Ulsan University Hospital, University of Ulsan College of Medicine, Ulsan, Korea.
  • 2Department of Pathology, Ulsan University Hospital, University of Ulsan College of Medicine, Ulsan, Korea.

Abstract

Von Hippel-Lindau disease (VHL) is a rare, autosomal dominantly transmitted familial, hereditary disease. This disease genetically predisposes affected people to the development of some types of tumor, such as cerebellar, spinal and medullary hemangioblastomas, and retinal angiomas, renal cell carcinomas and pheochromocytomas. Herein, a case of a cystic renal cell carcinoma, accompanied by Von Hippel-Lindau disease, treated by nephron sparing surgery, is reported. The VHL gene mutation of this case was also identified.

Keyword

Von Hippel-Lindau disease; Genes; Renal cell carcinoma

MeSH Terms

Carcinoma, Renal Cell*
Genetic Diseases, Inborn
Hemangioblastoma
Hemangioma
Nephrons*
Pheochromocytoma
Retinaldehyde
von Hippel-Lindau Disease*
Retinaldehyde
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