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Von Hippel–Lindau disease ( VHL ), also known as Von Hippel–Lindau syndrome, is a rare genetic disorder with multisystem involvement. [3] It is characterized by visceral cysts and benign tumors with potential for subsequent malignant transformation. It is a type of phakomatosis that results from a mutation in the Von Hippel–Lindau tumor ...
The most common cause of death in people with VHL disease is a type of slow-growing tumor in your central nervous system called hemangioblastoma. This type of tumor occurs in 13 to 72 percent of ...
The Von Hippel–Lindau tumor suppressor also known as pVHL is a protein that, in humans, is encoded by the VHL gene. Mutations of the VHL gene are associated with Von Hippel–Lindau disease, which is characterized by hemangioblastomas of the brain, spinal cord and retina. It is also associated with kidney and pancreatic lesions.
VHL normally functions as a tumor suppressor gene and thus when not functioning normally results in the development of benign and malignant tumors as well as cysts. Some of the most common manifestations include hemangioblastomas in the retina and central nervous system, clear cell renal cell carcinomas , pheochromocytomas , endolymphatic sac ...
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All-Russian Hockey League. The All-Russian Hockey League (VHL) [1] [2] (Russian: Всероссийская хоккейная лига (ВХЛ), Vserossiyskaya hokkeinaya liga (VHL) ), also known as the Major Hockey League [3] [4] or Higher Hockey League ( HHL ), [5] is a professional ice hockey league in Eurasia, and the second highest level ...
Hemangioblastoma are among the rarest central nervous system tumors, accounting for less than 2%. Hemangioblastomas usually occur in adults, yet tumors may appear in VHL syndrome at much younger ages. Men and women are approximately at the same risk.
Epidemiology. This is a very rare tumor, since only about 1 in 35,000 to 40,000 people have VHL, of whom about 10% have endolymphatic sac tumors. Patients usually present in the 4th to 5th decades without an gender predilection. The tumor involves the endolymphatic sac, a portion of the intraosseous inner ear of the posterior petrous bone.
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