PANCREATIC CYSTIC MANIFESTATIONS IN VON-HIPPEL-LINDAU-DISEASE

Conclusion. In view of the frequent absence of symptoms related to pan creatic lesions, screening tests for VHL should always include assessm ent of the pancreas and, considering the frequency of polycystic manif estations, VHL should always be borne in mind in the differential diag nosis of multiple pancreatic cysts, especially when occurring in young patients and in the absence of a positive history of pancreatic disea se, Background. Von Hippel-Lindau disease (VHL) is a hereditary diseas e transmitted with an autosomal dominant character and characterized b y hemangioblastomas of the central nervous system and retina, renal tu mors and cysts, and pheochromocytoma. Pancreatic manifestations of VHL are reported in the literature with incidences ranging from 16 to 29% of cases and consist mainly in cystadenomas of the serous type and in multiple cystic lesions, often with complete replacement of the gland . Methods and Results. We report five cases of VHL with a polycystic p ancreas as the main or only manifestation, all devoid of symptoms rela ted to involvement of the pancreas, who were referred to our Pancreati c Surgery center with diagnoses of multiple pancreatic pseudocysts of undefined origin.