Pancreatic manifestations of von Hippel-Lindau disease.
Von Hippel-Lindau disease is an uncommon, multisystem, multitumor disorder that can present in sporadic form but is more commonly inherited as an autosomal-dominant disease with high penetrance. Affected patients are at increased risk for developing multiple synchronous or metachronous benign or malignant, cystic, and vascular neoplasms of various organs. The characteristic neoplasms associated with von Hippel-Lindau are hemangioblastoma of the central nervous system and retina, clear cell renal cell carcinoma, and pheochromocytoma, but other lesions are well recognized. Pancreatic lesions, both primary and metastatic, are common, and several differential diagnostic possibilities must be considered.
Safo
Anthony-Osei F
AO
Department of Laboratory Medicine and Pathology, University of Minnesota Medical Center-Fairview, 420 Delaware Street SE, Minneapolis, MN 55455, USA.
Pambuccian
Stefan E
SE
D003937:Diagnosis, Differential; D020022:Genetic Predisposition to Disease; D006801:Humans; D010190:Pancreatic Neoplasms / Q000235:genetics* / Q000473:pathology* / Q000556:secondary / Q000628:therapy; D019683:Penetrance; D011379:Prognosis; D006623:von Hippel-Lindau Disease / Q000235:genetics*