von Hippel-Lindau (VHL) Disease
- 1 May 2008
- journal article
- review article
- Published by Wolters Kluwer Health in Advances in Anatomic Pathology
- Vol. 15 (3) , 165-171
- https://doi.org/10.1097/pap.0b013e31816f852e
Abstract
Von Hippel-Lindau (VHL) disease is an inherited multisystem familial cancer syndrome caused by mutations of the VHL gene on chromosome 3p25. A wide variety of neoplastic processes are known to be associated with VHL disease. The consequences of the VHL mutations and the pathway for tumor development continue to be elucidated. This paper will detail the variety of tumors associated with VHL disease and discuss the genetic mechanisms that lead to the predisposition for neoplasia.Keywords
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