Therapy of the Von-Hippel-Lindau syndrome
The cause of Von Hippel-Lindau syndrome is a mutation on chromosome three. A causal therapy is currently not possible. Therefore, only the option of symptomatic therapy remains.
Here, the size and localization of the vascular malformation are decisive. Small tumors in the area of the retina are treated by laser. This is one of the most successful therapies in which the patient’s vision is often largely preserved.
For larger angiomas, cryotherapy is used, for example. Here the tumor is frozen. Radiotherapy is also used, for example in the context of brachytherapy.
In this procedure, an enclosed radioactive source is placed in the immediate vicinity of the angioma. Malformations of the internal organs such as cysts on the liver, kidney and pancreas only need to be surgically addressed if they cause discomfort due to their size. Tumorous changes in the pancreas or kidney must be removed surgically. If the tumors are still small, surgery can often be performed to preserve the organs, so regular check-ups are essential.
Life expectancy of the Von-Hippel-Lindau syndrome
Von Hippel-Lindau syndrome is a genetic disorder with various benign and malignant malformations, particularly in the retina, cerebellum, kidney and adrenal gland. The prognosis of the patient depends on the size and location of the tumors. These are not equally pronounced in all patients.
Renal cell carcinoma is the most common cause of death. Vascular malformations in the brain can also lead to the patient’s death. The average life expectancy is given as about 50 years.
However, it must be said that through consistent check-ups, tumors are now detected and treated early. This leads to a significant improvement in life expectancy.
