Walsh & Hoyt: Von Hippel-Lindau Disease (Angiomatosis of the Retina and Cerebellum)

In 1904, the retinal lesions that characterize this entity were recognized by von Hippel and called ""angiomatosis retinae."" Lindau subsequently emphasized the similar pathologic nature between these retinal lesions and certain cerebellar tumors and also observed visceral tumors in the same patients. Although Lindau thought that the visceral lesions were benign, asymptomatic, and of no systemic significance, their potentially lethal nature was subsequently recognized, as was the hereditary nature of the disorder, which became known as von Hippel-Lindau disease (VHLD). This disorder continues to be the subject of intense genetic research.