The latter is an autosomal dominant hereditary neoplasia syndrome which is characterized by germline mutations of the vHL gene and by the predisposition to develop CNS or retinal HBL, renal cell carcinomas and cysts, pancreatic carcinomas and cysts, pheochromocytomas, and epididymal cystadenomas [32]. This evidence concerns the gene VHL and exocrine pancreatic carcinoma.