Figure 941

Von Hippel-Lindau disease (VHL): pancreas involvement. Contrast-enhanced abdominal CT in a patient with VHL shows multiple cysts in both pancreas (especially the tail, arrows) and kidneys. The majority of pancreatic cysts are asymptomatic. When they are numerous and large, they can induce diabetes mellitus or steatorrhea. Other, rare pancreatic lesions include microcystic adenoma, islet cell tumor, and carcinoma.

Study

Affected persons

Relatives at risk

Physical examination

Annual

Annual

24-h Urine collection for

Annual

Annual

metadrenaline and

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