VARIOUS PANCREATIC INVOLVEMENT IN VON HIP PEL-LINDAUS DISEASE

Von Hippel-Lindau's disease is a rate genetic disease, with an autosom al dominant merle of inheritance, characterised by the development of several tumours, such as haemangioblastoma of the central nervous syst em and retina, renal cysts or carcinoma and pheochromocytoma. Several pancreatic lesions, mainly represented by multiple cysts, are also enc ountered. We report here 8 cases of pancreatic involvement in patients affected with von Hippel-Lindau's disease. It consisted of multiple c ysts 13 cases), serous cystadenoma (2 cases), endocrine tumour (1 case ), haemangioblastoma (1 case) and ductal adenocarcinoma (1 case). Diag nosis of a rare lesion of the pancreas (multiple cysts, serous cystade noma or vascularized tumour) in a young patient may lead to search the other lesions of von Hippel-Lindau's disease and to undergo a familia l inquiry in order to propose a multidisciplinary approach for patient s affected by the disease.