Figure 937

Von Hippel-Lindau disease (VHL): central nervous system involvement. Gadolinium-enhanced brain magnetic resonance image of a patient with VHL, shows a typical cerebellar hemangioblastoma, appearing as a highly vascular nodule (arrow) in the wall of a cyst (arrowheads) located in the posterior fossa. Hemangioblastomas are benign tumors whose morbidity is due to mass effect. Cerebellar hemangioblastomas may present with symptoms of increased intracranial pressure. Spinal cord involvement may be manifested as syringomyelia. (Courtesy of S. Richard.)

FIGURE 9-38 (see Color Plate)

Von Hippel-Lindau disease (VHL): retinal involvement. Ocular fundus, A, and corresponding fluorescein angiography, B, in a patient with VHL, shows two typical retinal hemangioblastomas. The smaller tumor (arrow) appears at the fundus as an intense red spot, whereas the larger (arrow heads) appears as a pink-orange lake with dilated, tortuous afferent and efferent vessels. Small peripheral lesions are usually asymptomatic, whereas large central tumors can impair vision. (Courtesy of B. Snyers.)

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