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Upstate earns designation as a VHL Clinical Care Center

Upstate earns designation as a VHL Clinical Care Center

SYRACUSE, N.Y.-- An international organization that serves patients with an inherited disorder, called von Hipplel-Lindau or VHL, that causes tumors affecting the brain and other parts of the body, has designated Upstate Medical University as a VHL Clinical Care Center. Upstate is one of several centers in the United States and the only center in New York state outside of New York City to have earned this designation from the VHL Alliance.

With the designation as a Clinical Care Center, Upstate becomes an essential link in the chain of information for patients seeking treatment and for physicians worldwide who seek to provide the best care, according to VHL Alliance officials.

“Upstate now has become a tremendous resource for patients with hereditary syndromes and for those with associated kidney cancers or tumors,” said Gennady Bratslavsky, MD, chair of Upstate’s Department of Urology, who led the effort to gain the VHL designation. “We now get requests for evaluation of opinion from as far as California, and from patients traveling to Upstate from as far as Europe.”

Bratslavsky said that Upstate, as an academic and tertiary care center, promotes multidisciplinary approaches in managing complex cases, and that is precisely what is needed in managing patients with VHL.

Von Hippel-Lindau (VHL) is an inherited disorder causing multiple tumors, both benign and malignant, in the central nervous system (CNS) and in internal organs. The most common tumors are retinal and CNS haemangioblastomas, renal cell carcinoma (RCC), renal cysts and phaeochromocytoma.

Tumors can also arise in the pancreas, epididymis or broad ligament of the uterus, and the inner ear. The age at which the tumors present ranges from early childhood to the seventh decade of life. Early diagnosis, screening of family members and lifelong surveillance of VHL patients for tumors is recommended.

Although there is no cure for VHL, the associated tumors can be treated. Early detection and treatment of tumors significantly improves a patient’s diagnosis. Left untreated, VHL may result in blindness, permanent brain damage, metastatic cancer, or death.

Previously the average life expectancy for individuals with VHL was 50 years, where the main causes of death were renal cell carcinoma or central nervous system haemangioblastoma. Current screening protocols and treatment have improved this prognosis.

As a designated Clinical Care Center, Upstate provides significant patient and physician support and also ensures appropriate screening, according to guidelines offered by the VHL Alliance Clinical Advisory Council. Such screenings are recommended for individuals at risk of VHL who do not yet have symptoms or those who are known to have VHL, but have no symptoms. Screenings can start as early as child’s first year.

In order to be a designated center by the VHL, health care facilities must have a wide range of medical expertise available to care for patients. At Upstate, more than a dozen medical professionals from a range of specialties, including urology, hepatobiliary surgery, neurosurgery, neurology, otolaryngology, radiology, gynecology, ophthalmology, pediatrics, endocrinology, nephrology, dermatology as well as genetic counseling services, social work, and other supporting services are involved in the treatment of VHL patients.

In addition to VHL patients, several other syndromes associated with kidney cancers are evaluated at Upstate. “Since many genetic causes have recently been discovered for kidney cancer, the newly accredited VHL center will become a place for evaluation of most kidney cancer patients in the region,” Bratslavsky said.