Pathology

Treatment Options Ofr Von Hippel Lindau Syndrome



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Von Hippel Lindau Syndrome, named for the two doctors who discovered the syndrome, affects one in every 32,000 children born in the United States of America alone. The disease causes the infected person to produce tumors in areas of their body which contain large numbers of blood vessels; usually the retina, kidneys, pancreas, and the central nervous system. Although there is currently no way to cure a patient with Von Hippel Lindau Syndrome, there are a number of options for treatment.

There are a number of conditions a person suffering from Von Hippel Lindau Syndrome can have. Among them are kidney cancers, tumors of the central nervous system, and tumors or cysts of the pancreas. Because of the serious nature of this syndrome, it is extremely important for someone who has the syndrome to seek treatment from a variety of medical professionals who, as a team, are well versed in every aspect of this syndrome.

Treatment of Von Hippel Lindau Syndrome entails a combination of screening, and various therapies that must be given at specific times in order to be effective. Quality of life can be significantly improved if treatment is instituted in the very early stages of the syndrome.

Treatment of Pheochromocytoma

A pheochromocytoma is a rare tumor that can be found in the glands of the kidney region. It is most often treated with surgery. Although there are some instances in which part of the gland may not have to be removed, in most cases the entire gland is removed. However, there is a significant chance of increased blood pressure during surgery so a patient must take special medications for 2 -3 weeks prior to the day of the surgery. Additionally, if both glands are removed the patient will then have to undergo lifelong hormonal treatments.

Treatment of Hemangioblastoma

Hemangioblastomas are another potential issue with Von Hippel Lindau Syndrome. They are tumors that develop in part the central nervous system. The smaller ones that grow slowly will normally be monitored. However, the larger ones require surgery.

Treatment of Kidney Cancer

If suspicious masses found on the kidneys are of a certain size, the normal recommendation is removal. The same is true for fast-growing masses. In years past, if a patient had to have both kidneys removed they would be destined to undergo lifelong dialysis treatments. Advances in medicine have made it possible to potentially remove only some of the kidney, leaving enough of the organ for normal function — at least for a while.

When the cancer has spread farther than the kidney, current treatment includes immunotherapy and chemotherapy. New forms of therapy are currently being developed that will hopefully allow a patient with Von Hippel Lindau Syndrome to seek treatment that is not so toxic and is more effective.

Treatment of Ocular Tumors

As with all the other manifestations of this syndrome, it is critical for patients suffering from ocular tumors to seek early diagnosis. Failure to do so could result in permanent loss of vision. Laser treatment or cryotherapy (a treatment that uses extremely cold probes that serve to destroy the tumor) is used for tumors located on the outer regions of the retina. Patients who suffer from detached retina or scarring of the retina usually undergo surgery. Tumors in the most delicate areas of the eye are usually not treated with surgery because the procedure is so invasive and damaging in nature.

Treatment of Pancreatic Cysts and Cystadenomas

These types of cysts are not usually treated with surgery. If the cysts grow extremely large, fluid from the cysts are usually extracted to diminish their size.

Tumors of the pancreas have the extreme potential to spread, which could have fatal results, so these tumors are monitored very closely. If the tumor reaches a certain size, it is usually surgically removed.

Treatment for Von Hippel Lindau Syndrome is critical. If you or someone you know suffers from Von Hippel Lindau Syndrome make certain you or they are receiving the best treatment possible.


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