<h3>What is Von Hippel-Lindau disease (VHL)?</h3> <p>Von Hippel-Lindau disease (VHL) is a rare disease that causes tumors and cysts to grow in your body. They can grow in your brain and spinal cord, kidneys, pancreas, adrenal glands, and reproductive tract. The tumors are usually benign (non-cancerous). But some tumors, such as those in the kidney and pancreas, can become cancerous.</p> <h3>What causes Von Hippel-Lindau disease (VHL)?</h3> <p>VHL is a genetic disease. It is inherited, which means that it is passed down from parent to child.</p> <h3>What are the symptoms of Von Hippel-Lindau disease (VHL)?</h3> <p>Symptoms of VHL depend on the size and location of the tumors. They may include:</p><ul> <li><a href="https://medlineplus.gov/headache.html">Headaches</a></li> <li>Problems with <a href="https://medlineplus.gov/balanceproblems.html">balance</a> and <a href="https://medlineplus.gov/walkingproblems.html">walking</a></li> <li><a href="https://medlineplus.gov/dizzinessandvertigo.html">Dizziness</a></li> <li>Weakness of the limbs</li> <li><a href="https://medlineplus.gov/visionimpairmentandblindness.html">Vision problems</a></li> <li><a href="https://medlineplus.gov/highbloodpressure.html">High blood pressure</a></li> </ul> <h3>How is Von Hippel-Lindau disease (VHL) diagnosed?</h3> <p>Detecting and treating VHL early is important. Your health care provider may suspect that you have VHL if you have certain patterns of cysts and tumors. There is a genetic test for VHL. If you have it, you will need other tests, including imaging tests, to look for tumors and cysts.</p> <h3>What are the treatments for Von Hippel-Lindau disease (VHL)?</h3> <p>Treatment can vary, depending on the location and size of the tumors and cysts. It usually involves surgery. Certain tumors may be treated with radiation therapy. The goal is to treat growths while they are small and before they do permanent damage. You will need to have careful monitoring by a doctor and/or medical team familiar with the disorder.</p> <p class="">NIH: National Institute of Neurological Disorders and Stroke</p>

Von Hippel-Lindau syndrome is an inherited disorder characterized by the formation of tumors and fluid-filled sacs (cysts) in many different parts of the body. Tumors may be either noncancerous or cancerous and most frequently appear during young adulthood; however, the signs and symptoms of von Hippel-Lindau syndrome can occur throughout life.~Tumors called hemangioblastomas are characteristic of von Hippel-Lindau syndrome. These growths are made of newly formed blood vessels. Although they are typically noncancerous, they can cause serious or life-threatening complications. Hemangioblastomas that develop in the brain and spinal cord can cause headaches, vomiting, weakness, and a loss of muscle coordination (ataxia). Hemangioblastomas can also occur in the light-sensitive tissue that lines the back of the eye (the retina). These tumors, which are also called retinal angiomas, may cause vision loss.~People with von Hippel-Lindau syndrome commonly develop cysts in the kidneys, pancreas, and genital tract. They are also at an increased risk of developing a type of kidney cancer called clear cell renal cell carcinoma and a type of pancreatic cancer called a pancreatic neuroendocrine tumor.~Von Hippel-Lindau syndrome is associated with a type of tumor called a pheochromocytoma, which most commonly occurs in the adrenal glands (small hormone-producing glands located on top of each kidney). Pheochromocytomas are usually noncancerous. They may cause no symptoms, but in some cases they are associated with headaches, panic attacks, excess sweating, or dangerously high blood pressure that may not respond to medication. Pheochromocytomas are particularly dangerous in times of stress or trauma, such as when undergoing surgery or in an accident, or during pregnancy.~About 10 percent of people with von Hippel-Lindau syndrome develop endolymphatic sac tumors, which are noncancerous tumors in the inner ear. These growths can cause hearing loss in one or both ears, as well as ringing in the ears (tinnitus) and problems with balance. Without treatment, these tumors can cause sudden profound deafness.~Noncancerous tumors may also develop in the liver and lungs in people with von Hippel-Lindau syndrome. These tumors do not appear to cause any signs or symptoms.