Von Hippel-Lindau syndrome

Definition

Von Hippel-Lindau (VHL) syndrome is an inherited condition characterized by tumors that arise in multiple locations in the body. Some of these tumors cause cancer and some do not. Many of the tumors seen in VHL are vascular, meaning that they have a rich supply of blood vessels.

Description

In the mid-1800s, ophthalmologists described vascular tumors in the retina, the light-sensitive layer that lines the interior of the eye. These tumors, called angiomas, were not cancerous but were associated with vision loss. In 1904, a German ophthalmologist named Eugen von Hippel noted that these retinal angiomas seemed to run in families. Twenty-three years later, Arvid Lindau, a Swedish pathologist, reported a...

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