Some of these tumors are benign, while others are cancerous. Early detection and careful monitoring are particularly important for these organ systems, usually with CT, MRI, or ultrasound scanning yearly. Von Hippel-Lindau is a genetically transmitted condition. It is caused by a dominant gene. Even in people who have this gene, however, there is a wide variation in the date of onset of the disease, the organ system in which the problem occurs, or the severity of the involvement.
Every person is different.
The VHL Family Alliance has prepared a booklet which discusses VHL in greater detail, and helps to explain some of the medical terminology medical professionals may use in discussing diagnosis and treatment. Each possible evidence of the disease has its own diagnostic evaluation. If someone has a family history of VHL, it is important to begin screening early, before any symptoms occur. The doctor should be consulted about the best time to begin screening, and the right schedule for return visits. It is generally recommended to begin regular screening at age six, or even earlier.