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von Hippel-Lindau (VHL) Disease

2008; Lippincott Williams & Wilkins; Volume: 15; Issue: 3 Linguagem: Inglês

10.1097/pap.0b013e31816f852e

1533-4031

Bahig M. Shehata, Christina A. Stockwell, Amilcar A. Castellano‐Sanchez, Shannon V. Setzer, Christine Schmotzer, H. B. Robinson,

RNA modifications and cancer

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.