First, a National Familial Pancreas Tumor Registry (NFPTR; http://pathology.jhu.edu/pancreas/nfptr/index.php) analysis of the germline DNA of 638 familial pancreatic cancer patients from 593 kindreds revealed that germline pathogenic variants of ATM, BRCA2, CDKN2A, and PALB2 elevated the risk of pancreatic cancer, and that variants of BUB1B, CPA1, FANCC, and FANCG are detected more frequently in familial than in sporadic pancreatic cancer [37]. This evidence concerns the gene CPA1 and familial pancreatic carcinoma.