Von hippel-lindau syndrome

Treatment

What are the first steps after an initial diagnosis of von Hippel-Lindau syndrome?

After an initial diagnosis of von Hippel-Lindau (VHL) syndrome, your doctor will take a comprehensive medical history to look for signs and symptoms of disease and will also run tests to determine whether specific organs are affected by tumors or cysts. You may be referred to an eye specialist (ophthalmologist), a specialist who assesses and treats disorders of the brain and spinal cord (neurologist), a cancer specialist (oncologist), and other specialists or healthcare professionals.

The best way to obtain the most accurate, current, clear, and comprehensive information is to be seen at a VHL Clinical Care Centers. These are genetic centers that specialize in the treatment of patients with VHL syndrome. At most centers you will see a medical geneticist, genetic counselor, and nurse who work as a team to answer your questions, discuss testing, identify your at-risk family members, and develop a comprehensive evaluation and treatment plan for you. A Center will work with your current doctors to organize the treatment, tests, and specialists you need. The VHL Alliance has information on Clinical Care Centers. The genetic counselor will also review family history and identify other family members who would benefit from genetic testing for VHL. To find a genetic counselor near you who can discuss testing and results, search on the National Society of Genetic Counselor's website.your area.

References
  • Von Hippel-Lindau syndrome. Genetics Home Reference website. Accessed September 8, 2016. https://ghr.nlm.nih.gov/condition/von-hippel-lindau-syndrome
  • VHL Alliance website. https://vhl.org/
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More Treatment Content

Is there a treatment for von Hippel-Lindau syndrome?

How are hemangioblastomas in von Hippel-Lindau syndrome treated?

How is renal cell carcinoma treated in von Hippel-Lindau syndrome?

How are pheochromocytomas treated in von Hippel-Lindau syndrome?

Is surveillance important in von Hippel-Lindau syndrome?

Is there a treatment for von Hippel-Lindau syndrome?

There is no cure for von Hippel-Lindau (VHL) syndrome in 2019. There are several treatments used to help reduce or manage the health problems seen in VHL syndrome. However, because the organ systems involved can vary greatly among people with this disorder, the treatment options will be very different as well. This can be true even for people in the same family. Early detection and prompt treatment of tumor or cysts is extremely important. People with VHL syndrome must be routinely evaluated by doctors so that any new tumor or cyst formation can be detected immediately and treated appropriately. This is called surveillance. People with VHL syndrome will require lifelong surveillance of their disorder.

References
  • Frantzen C, Klasson TD, Links TP, Giles RH. Von Hippel-Lindau syndrome. GeneReviews website. Accessed September 8, 2016. http://www.ncbi.nlm.nih.gov/books/NBK1463/
How are hemangioblastomas in von Hippel-Lindau syndrome treated?

Surgery is the main way hemangioblastomas of the brain and spinal cord are treated in von Hippel-Lindau (VHL) syndrome. Surgery is only used when a hemangioblastoma is causing symptoms or growing rapidly. These tumors are not cancerous, but they can cause problems by pressing against or damaging nearby organs and tissues. Small growths that do not cause symptoms and are not growing may not require treatment. Instead, doctors will routinely check these growths and if they begin to grow may recommend treatment. Researchers are studying different drugs that they hope will treat the different symptoms of VHL syndrome; these drugs are currently in clinical trials.

Hemangioblastomas in the eyes can be treated with lasers, which are used to destroy the tumor or cyst. Sometimes, doctors may recommend cryotherapy. This is when doctors freeze the tumor or cyst. After the tumor or cyst thaws out, the tissue usually disintegrates.

Talk to your doctor and entire medical team about treatment options that may be appropriate in your situation.

References
  • Cryosurgery in Cancer Treatment. National Cancer Institute website. http://www.cancer.gov/about-cancer/treatment/types/surgery/cryosurgery-fact-sheet
  • Plon SE, Jonasch E. Clinical features, diagnosis, and management of von Hippel-Lindau disease. UpToDate, Inc.
How is renal cell carcinoma treated in von Hippel-Lindau syndrome?

As many as 70% of people with von Hippel-Lindau (VHL) syndrome will develop a form of kidney cancer called renal cell carcinoma. Treatment of this type of carcinoma involves an individualized plan that may include surgery, radiation, chemotherapy, or other therapies. Surgical techniques used will attempt to remove the growth while sparing as much of the kidney as they can. This is done to preserve kidney function for as long as possible. Although uncommon, some people may also have a kidney transplant; this is usually done when cancer affects both kidneys or the kidneys are failing.

Talk to your doctor and entire medical team about treatment options that may be appropriate in your situation.

References
  • Plon SE, Jonasch E. Clinical features, diagnosis, and management of von Hippel-Lindau disease. UpToDate, Inc.
How are pheochromocytomas treated in von Hippel-Lindau syndrome?

Some people with von Hippel-Lindau (VHL) syndrome develop pheochromocytomas. This is a tumor that usually forms in the adrenal glands (although they can be found in other areas of the body). A pheochromocytoma may not cause symptoms or it can cause high blood pressure, excessive sweating and heart palpitations. If a doctor feels treatment is required, surgery is the main therapy. Before surgery, it is extremely important the people receive drugs that block the effects of the adrenal glands.

Talk to your doctor and entire medical team about treatment options that may be appropriate in your situation.

References
  • Plon SE, Jonasch E. Clinical features, diagnosis, and management of von Hippel-Lindau disease. UpToDate, Inc.
  • Pheochromocytoma. The National Organization for Rare Disorders website. http://rarediseases.org/rare-diseases/pheochromocytoma/
Is surveillance important in von Hippel-Lindau syndrome?

Individuals with von Hippel-Lindau (VHL) syndrome should undergo lifelong surveillance. This is done to detect any cysts or tumors as early as possible so they can be treated promptly. A surveillance plan may include annual eye exams, MRIs of the head to evaluate the brain, MRIs of the spine, screening for renal cell carcinoma, screening for pancreatic cysts or tumors, and screening for pheochromocytoma including regular testing for high blood pressure. Hearing should be periodically checked as well. Your doctor and entire medical team will work together to develop a comprehensive surveillance plan for VHL syndrome.

The VHL Alliance provides information on the Importance of Surveillance.

References
  • Maher ER, Neumann HPH, Richard S. von Hippel-Lindau disease: A clinical and scientific review. Eur J Hum Genet. 2011;19:617-623.
  • VHL Alliance website. https://vhl.org/professionals/screening-diagnosis/importance-of-screening/

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