von Hippel-Lindau Disease Treatment
Because von Hippel-Lindau disease (VHL) can cause several 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 a commonly used treatment for many 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 VHL disease each year, using the least invasive and most advanced techniques.
A new targeted therapy is also available patients with VHL-related hemangioblastomas and of the brain and spine, kidney cancer and pancreatic neuroendocrine tumors. This promising therapy may allow some patients to avoid surgery for specific tumors.
Our von Hippel-Lindau disease treatments
If you are diagnosed with VHL, your treatment will depend on locations of the tumors and cysts that develop and the problems they cause.
Since surgery is one of the primary treatments for the disease, doctors will often try to coordinate surgeries for different growths. This limits the number of procedures a patient undergoes, reducing recovery time and the trauma of surgery on the body.
Endolymphatic sac tumor treatment
Endolymphatic sac tumors are removed through minimally invasive surgery. If surgery is not possible, these tumors are treated with radiation therapy.
Hemangioblastoma treatment
If hemangioblastomas are small and/or slow-growing, they may not need treatment. Tumors that are large, fast-growing or symptom-causing are surgically removed or may in some cases be treated with focused-beam or stereotactic radiation therapy.
In addition to surgery, a newly developed targeted therapy is available to VHL patients with hemangioblastoma of the brain and spine. This therapy may help patients avoid surgery. Doctors are studying how to best integrate this targeted therapy into hemangioblastoma treatment plans.
Retinal hemangioblastomas 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.
Kidney cancer treatment
Kidney tumors usually are surgically removed when they grow larger than three 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
In addition to surgery, a newly developed targeted therapy is available to VHL patients with kidney cancer. This therapy may help patients avoid surgery. Doctors are studying how to best integrate this targeted therapy into kidney cancer treatment plans.
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. Learn more about kidney cancer treatment.
Pancreatic tumors and cysts treatment
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 (NET) becomes larger than 3 centimeters, it is surgically removed.
In addition to surgery, a newly developed targeted therapy is available to VHL patients with pancreatic NETs. This therapy may help patients avoid surgery. Doctors are studying how to best integrate this targeted therapy into pancreatic NET treatment plans.
Pheochromocytoma treatment
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.
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. Learn more about clinical trials.
