Pancreatic manifestations of von Hippel-Lindau disease-effect of imaging on clinical management.

OBJECTIVE: To assess the impact of imaging on pancreatic lesion management in von Hippel-Lindau disease (VHL).

METHODS: We reviewed sequential computed tomography (CT) and magnetic resonance examinations (1997-2008) of 33 patients with VHL who had at least 1 pancreatic lesion.

RESULTS: One hundred sixty-seven imaging studies demonstrated innumerable simple pancreatic cysts and 58 complex pancreatic masses: 24 were complex cystic and 34 were solid (30 small [<or=2 cm] and 4 large [>2 cm]). Aggregate annual growth was significant in complex cystic and solid masses (mean, 0.39 cm/y [P = 0.006] and 0.14 cm/y [P = 0.045]). Solid mass growth differed by size (small: 0.06 cm/y [range, -0.09 to 0.31 cm/y]; large: 1.28 cm/y [range, 0-1.98 cm/y]). Thirteen masses were excised. No patient developed metastases. Arterial-phase CT improved (P = 0.0003) solid mass detection, but 28% of studies still underreported the total number.

CONCLUSIONS: Most pancreatic masses in VHL do not require annual surveillance. Arterial-phase CT improves mass detection, but many masses remain prospectively missed.