children s health issues
Von Hippel–Lindau Disease (VHL)
Treatment of VHL
Surgery or sometimes radiation therapy For angiomas of the retina, laser therapy or application of extreme cold Sometimes the medicationbelzutifan Tumors are surgically removed, if possible, before they cause permanent damage. High-dose radiation therapy, focused on the tumor, can sometimes be used instead. People who have tumors in the adrenal glands may also need medications to control their blood pressure. People who have advanced kidney cancer may be given other medications. Belzutifanmay be used in adults who have kidney cancer, tumors in the brain or spine, or tumors in the pancreas that do not need to be immediately surgically removed. This medication shrinks tumors and stops them from progressing. It can be used until the disease worsens or until any side effects become too severe. Typically, angiomas of the retina are destroyed using laser therapy or application of extreme cold (cryotherapy). These procedures help preserve vision. Because new complications and tumors can develop in von Hippel-Lindau disease, affected people must be closely monitored for the rest of their life. In people who have been diagnosed with von Hippel-Lindau disease, the following screening examinations and tests are repeated every 1 or 2 years and when symptoms occur: Physical examination Eye examination to screen for eye tumors Blood pressure checks and urine and blood tests to screen for Pheochromocytoma None Magnetic resonance imaging (MRI) of the brain and spine to screen for tumors Hearing tests to screen for inner ear tumors MRI or ultrasonography of the abdomen to screen for kidney cancer, pheochromocytomas, and pancreas tumors In people who have not been diagnosed with von Hippel-Lindau disease but who have the abnormal gene or who have not been tested but who have a parent or sibling with von Hippel-Lindau disease, blood pressure monitoring and examinations to look for vision, hearing, and eye problems are done every year. 