Difficult Diagnosis of Atypical Cystic Pancreatic Lesions in von Hippel-Lindau Disease

von Hippel-Lindau disease is a progressive, autosomal dominant disorder with multiorgan involvement. There are 2 types of pancreatic lesions from von Hippel-Lindau disease: cystic lesions and endocrine pancreatic tumors. Only the latter type is potentially malignant and may justify pancreatic resection. The differential diagnosis between these 2 types of lesions can be difficult. We report 3 patients with atypical cystic pancreatic lesions who underwent surgery for suspected malignant tumors. The pathological-radiological correlations and the diagnostic and management strategies are discussed. Abbreviations: VHL-von Hippel-Lindau disease, EPTs-endocrine pancreatic tumors, MDCT-multidetector computed tomography, MR-magnetic resonance imaging, EUS-endoscopic ultrasonography, NSE-neuron-specific enolase, SRS-somatostatin receptor scintigraphy, CEA-carcinoembryonic antigen, CA 19.9-carbohydrate antigen 19.9, CA 72.4-carbohydrate antigen 72.4