# What is Von Hippel-Lindau (VHL) disease? Canonical page: https://clinicsjo.com/en/faq/ma-howa-marad-fon-hibl-lindaw-vhl Language: en Category: oncology Publisher: ClinicsJo Published: 2026-09-13T08:09:53.368Z Content updated: 2026-09-13T08:09:53.368Z Translation (ar): https://clinicsjo.com/ar/faq/ma-howa-marad-fon-hibl-lindaw-vhl Von Hippel-Lindau (VHL) disease is a rare genetic condition characterized by the formation of tumors and fluid-filled or air-filled cysts in various parts of the body. These tumors can be benign (non-cancerous) or malignant (cancerous), and while they often appear in young adulthood, signs and symptoms can emerge at any stage of life [\[1\]](). Many organs are affected by VHL, including the brain, spinal cord, retina, inner ear, adrenal glands, kidneys, and pancreas [\[1\]](). Many people with VHL live long lives, especially when tumors and cysts are detected, monitored, and treated early [\[1\]](). ## Common Tumors and Cysts in VHL Common tumors and cysts associated with VHL include: - **Hemangioblastomas:** These are non-cancerous tumors consisting of many blood vessels. They often appear in the brain, spinal cord, or eyes and can grow unpredictably. If they become large, they may compress the brain or spinal cord and affect functions such as vision, hearing, or fluid flow in the nervous system [\[1\]](). - **Kidney tumors:** People with VHL face an increased risk of developing a type of kidney cancer called clear cell renal cell carcinoma (RCC) [\[2\]](). - **Pancreatic tumors:** Cysts may form in the pancreas, and there is also a risk of developing a type of pancreatic cancer called pancreatic neuroendocrine tumor [\[2\]](). - **Pheochromocytoma:** This tumor often occurs in the adrenal glands (small, hormone-producing glands above the kidneys). Typically, pheochromocytoma is non-cancerous and may not cause symptoms, but in some cases, it may be associated with headaches, panic attacks, excessive sweating, or dangerous high blood pressure that may not respond to medication [\[2\]](). - **Endolymphatic Sac Tumors:** About 10% of people with VHL develop these non-cancerous tumors in the inner ear. They can cause hearing loss in one or both ears, as well as tinnitus and balance problems [\[2\]](). ## Causes of Von Hippel-Lindau Disease VHL disease is caused by mutations in the VHL gene [\[1\]](). This gene is responsible for producing a protein that suppresses tumor formation. When this protein does not function properly, tumors can form more easily in the body [\[1\]](). VHL syndrome is inherited in an autosomal dominant pattern, meaning a person only needs to inherit one copy of the mutated gene from one parent to develop the syndrome [\[1\]](). In about 20% of cases, the mutation is new and not inherited [\[3\]](). ## Symptoms of Von Hippel-Lindau Disease Symptoms of VHL vary from person to person and depend on the size and location of the tumors or cysts. Symptoms may include [\[1\]](): - Headaches - Balance and walking problems - Dizziness - Weakness in an arm or leg - Vision problems - Hearing problems in one or both ears - High blood pressure - Vomiting ## Next Steps and Emergency Care If you suspect that you or a family member may have VHL, it is important to talk to your doctor about genetic testing [\[1\]](). Early diagnosis and regular medical follow-up are essential to monitor tumors and cysts and treat them in their early stages, which improves treatment outcomes [\[1\]](). Treatment may include surgery, radiation therapy, or medications like belzutifan (Welireg), which can slow tumor growth and prevent the formation of new tumors [\[1\]](). In the event of acute symptoms such as sudden severe headache, frequent vomiting, sudden weakness in the limbs, or severe vision or balance problems, emergency care should be sought immediately, as these symptoms may indicate pressure on the brain or spinal cord requiring urgent intervention [\[1\]](). [Treatment options for Von Hippel-Lindau disease (Arabic)]() can vary and depend on the individual case. ## Medical references - [Von Hippel-Lindau (VHL) Syndrome]() - [Von Hippel-Lindau syndrome]() - [Von Hippel-Lindau syndrome: MedlinePlus Genetics]() General health education; not a substitute for individualized clinical assessment. Citation URL: https://clinicsjo.com/en/faq/ma-howa-marad-fon-hibl-lindaw-vhl#answer