[Familial pancreatic carcinoma]

Abstract

Less than 10% of pancreatic ductal adenocarcinomas are based on a hereditary syndrome. In contrast a positive family history for pancreatic cancer raises the individual risk for the development of pancreatic and extrapancreatic malignancies.While 70% of hereditary pancreatic carcinomas can be attributed to the familiar pancreatic cancer syndrome 30% are caused by other hereditary syndromes, e.g., Peutz-Jeghers syndrome or familial adenomatous polyposis. Furthermore, genetically determined pancreatitis (e.g., hereditary pancreatitis or cystic fibrosis) can lead to pancreatic cancer.Up to now conclusive data for routine screening of high risk patients are not available which is due in part to the difficult identification of high risk patients and the problematic classification of detected pancreatic lesions. Therefore, high risk patients should be included in controlled clinical trials for screening. Small pancreatic lesions are not clear indications for surgical resection as false positive results can hamper a clear diagnosis and prophylactic pancreatectomy is not recommended. In the case of a histologically proven carcinoma a prophylactic extension of resection might be reasonable. Prevention of familial pancreatic cancer can be achieved through nicotine abstinence.