Brain and spine
Tumours known as
hemangioblastomas can form in the blood vessels of the brain and along the spinal cord. While these tumours are non-cancerous, they can cause symptoms depending on where they are located.
Cysts in the kidneys are also common in individuals with VHL. Individuals are also at increased risk of developing a type of kidney cancer called clear cell renal cell carcinoma.
Individuals with VHL may develop cysts in their pancreas which are non-cancerous. A tumour called
a pancreatic neuroendocrine tumour (pNET) can also develop in the pancreas. These tumours can be benign (non-cancerous) or malignant (cancerous).
Benign tumours/cysts called cystadenomas can develop in the reproductive tract of both females and males. These tumours or cysts can cause discomfort or pain and surgery can be considered to remove them.
Tumours can occur at the back of the retina and are called retinal angiomas. These tumours can lead to problems with vision or even blindness if not treated. Symptoms affecting vision usually present early in life.
Non-cancerous tumours can form in the endolymphatic duct of the inner ear, which regulates the balance of an individual. These tumours are called
endolymphatic sac tumours, which can cause problems such as hearing loss or deafness. Other symptoms include ringing in the ears, balance problems and dizziness.
Adrenal glands are found on top of the kidneys and are involved in hormone regulation. A type of tumour called a
phaeochromocytoma can occur within the adrenal glands. While these tumours are usually non-cancerous, they can cause symptoms such as high blood pressure, headaches, excessive sweating and a fast heart rate, which can lead to other more serious medical complications (such as a heart attack) if undetected and untreated.
It is important to seek prompt medical action when you experience any of the above symptoms associated with VHL, as it will help to minimise irreversible complications and also to ensure you receive screening for the early detection of any tumours and cancers.
Individuals with VHL can develop tumours and cysts in various parts of their body, such as their eyes, ears, brain, spinal cord, kidneys, adrenal glands, pancreas and reproductive tract.
Note: The conditions associated with a faulty VHL gene and their risk estimates may change as more information is available.
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