Familial Pancreatic Cancer

Abstract

Familial pancreatic cancer accounts for 10% of all patients with pancreatic cancer. Because the 5-year survival rate of pancreatic cancer is only 7%, screening programs for high-risk individuals are essential and might be advantageous. Pancreatic ductal adenocarcinoma mostly shows symptoms at an advanced state and treatment is not efficient enough to cure most patients. People with hereditary tumor syndromes or their affected relatives can also be included in such screening programs. Besides the collection of data to investigate the background of the disease, these screening programs aim to diagnose and treat precursor lesions so that more dangerous, invasive lesions are prevented. These precursor lesions can be pancreatic intraepithelial neoplasia, intraductal papillary mucinous neoplasm, and mucinous cystic neoplasm. This review summarizes the latest knowledge of pancreatic screening programs, shows the procedure of pancreatic cancer screening, and gives an overview of current guidelines.

Keywords: Familial pancreatic cancer; Hereditary tumor syndromes; High-risk individuals; International Cancer of the Pancreas Screening Consortium; Intraductal papillary mucinous neoplasm; Mucinous cystic neoplasm; Pancreatectomy; Pancreatic intraepithelial ­neoplasia; Peutz-Jeghers syndrome; Screening.