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Von Hippel-Lindau disease
Background
Von Hippel-Lindau (VHL) disease is an uncommon genetic disorder in which tumours and cysts occur in a variety of organs. Most frequently these are the cerebellum (hind part of the brain), the spinal cord, the kidneys, the pancreas (a gland situated below the stomach) and the retina (a light-sensitive film at the back of the eye). Angiomas (enlarged blood vessels) can occur on the retina, while haemangioblastomas (benign(non-cancerous) cystic or solid tumours) may occur in the cerebellum or spinal cord. Kidney tumours and cysts are common and early detection of kidney tumours is important as these can become cancerous. Phaeochromocytomas, tumours of the inner part of the adrenal gland called phaeochromocytoma, occur in about ten per cent of patients, pancreatic tumours in up to ten per cent and endolymphatic sac tumours (part of the inner ear) causing deafness occur in up to five per cent of patients. Cysts in the pancreas and (in males) the epididymis (a narrow, tightly-coiled tube behind the testicle) are common, but rarely cause clinical problems.
