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5 Facts you should know about

Von Hippel-Landau disease

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1

VHL disease is a rare systemic syndrome that can cause recurrent tumor development in the kidneys, CNS, and pancreas

2

It is a multi-organ, autosomal dominant condition characterized by abnormal cell growth and caused by genetic aberrations of the tumor suppressor gene VHL

3

Tumors that develop are often benign but may cause symptoms, depending on location, as the tumor grows. Some tumors, however, may become cancerous, such as those found in the kidneys and pancreas

4

As many as 70% of people with VHL disease develop renal cell carcinoma by 60 years of age - RCC begins to appear at a median age of 31 years

5

Lack of functional VHL protein results in accumulation of HIF-2α

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