<prompt> A 3 year old child develops headaches and is brought to the family doctor. Funduscopic examination reveals papilledema; one retina also shows a very vascular tumor. CT of the head demonstrates a cystic tumor of the cerebellum. This child has a high likelihood of later developing which of the following?Berry aneurysm of the basilar systemBilateral renal cell carcinomaCancer of a peripheral nerveChoreiform movements related to decreased GABA and acetylcholine  <response>: The disease is von Hippel-Landau disease, which is associated with a deletion involving the VHL gene on chromosome 3 3p. Affected individuals develop vascular tumors hemangioblastomas of the retina, cerebellum, andor medulla. Roughly half of the affected individuals later develop multiple, bilateral renal cell carcinomas. Berry aneurysms are unrelated to hemangioblastomas, but are instead associated with adult polycystic disease. Peripheral nerve cancers are a feature of von Recklinghausens disease neurofibromatosis type I. Choreiform movements, related to decreased GABA and acetylcholine, are a feature of Huntingtons disease. Ref: Wyatt C., Butterwoh IV J.F., Moos P.J., Mackey D.C., Brown T.G. 2008. Chapter 6. Genetic Disorders. In C. Wyatt, J.F. Butterwoh IV, P.J. Moos, D.C. Mackey, T.G. Brown Eds, Pathology: The Big Picture. 