Our Treatment Approach
Because VHL has the potential to cause a number of different problems, MD Anderson brings together a team of experts from several disciplines to treat the disease. They work together to personalize your care, ensuring the most-advanced and most-effective treatments possible.
Surgery, including minimally invasive and laser surgery in some cases, is the main treatment for VHL-associated problems. Like all surgeries, surgery for VHL disorders is most successful when performed by a specialist with a great deal of experience in the particular procedure.
MD Anderson’s surgeons are among the most skilled and renowned in the world. They perform a large number of surgeries for von Hippel Lindau disease each year, using the least-invasive and most-advanced techniques.
Our von Hippel Lindau Disease Treatments
If you are diagnosed with VHL, your treatment will depend on the problems it causes.
Surgery is the main treatment for pheochromocytomas. To prevent complications from severe high blood pressure caused by high catecholamine levels during surgery, you must take medicine for two to three weeks before the procedure.
Removal of a pheochromocytoma may require taking out the whole adrenal gland. This is called an adrenalectomy. Sometimes the surgeon can “spare” the cortex (outer layer) by removing only the tumor and the adrenal medulla. Sometimes, minimally invasive laparoscopic surgery, which uses a smaller incision and may help you recover quicker, is possible.
If both adrenal glands are removed, you will need to take medicine to give your body the hormones that usually are made by the adrenal gland.
If hemangioblastomas are small and/or slow-growing, they may not need treatment. Your doctor will observe the tumor and determine if you surgery is necessary. Tumors that are large, fast growing or symptom causing are surgically removed or treated with focused-beam or stereotactic radiation therapy.
Kidney tumors usually are surgically removed when they grow larger than 3 centimeters in diameter or if they grow quickly. In addition, MD Anderson offers advanced treatments that can help some people, including:
- Partial nephrectomy, surgical removal of part of the kidney
- Radiofrequency ablation, which uses radiofrequency waves to destroy the tumor
- Cryoablation, which uses extreme cold to remove tissue
If kidney cancer spreads beyond the kidney, it becomes challenging to treat. Immunotherapy and chemotherapy help some people. New therapies include anti-angiogenesis (blood vessel starving) medications, which stop blood vessels from growing, and targeted therapies that help your body fight the disease on a molecular level.
It is important that eye problems caused by VHL receive early diagnosis and treatment. This helps lower the chance for vision loss.
Tumors found on the outer regions of the retina can be treated with laser photocoagulation (treatment with laser beams that heat and destroy tumors), or cryotherapy (treatment with extremely cold probes that destroy the tumor). Vitrectomy, or surgical treatment, should be considered for patients with detachment and/or scarring of the retina.
Tumors in very delicate areas, like the optic disc, usually are not treated because of the damage treatment might cause.
Pancreatic Cysts and Cystadenomas
Cysts and cystadenomas in the pancreas generally do not require treatment. Rarely, if a cyst becomes very large, your doctor may shrink it by using a needle to draw fluid from the cyst.
Pancreatic neuroendocrine tumors require close follow up because they may spread, which can be very dangerous. Generally, if a known or suspicious pancreatic neuroendocrine tumor becomes larger than 3 centimeters, it is surgically removed.
Our von Hippel Lindau Disease Clinical Trials
As one of the nation’s leading research centers, MD Anderson offers clinical trials of new treatments for von Hippel Lindau disease.