ReviewsInherited Pancreatic Cancer SyndromesSolomon, Sheila MS, CGC; Das, Siddhartha BS; Brand, Randall MD; Whitcomb, David C. MD, PhDAuthor Information From the *Division of Gastroenterology, Departments of Medicine, †Human Genetics, and ‡Cell Biology and Molecular Physiology, University of Pittsburgh, Pittsburgh, PA. Reprints: David C. Whitcomb, MD, PhD, Department of Cell Biology and Molecular Physiology, University of Pittsburgh, Room 401.4, 3708 Fifth Ave, Pittsburgh, PA 15213. E-mail: [email protected]. The Cancer Journal: November/December 2012 - Volume 18 - Issue 6 - p 485-491 doi: 10.1097/PPO.0b013e318278c4a6 Buy Metrics Abstract Pancreatic cancer remains one of the most challenging of all cancers. Genetic risk factors are believed to play a major role, but other than genes coding for blood group, genetic risks for sporadic cases remain elusive. However, several germline mutations have been identified that lead to hereditary pancreatic cancer, familial pancreatic cancer, and increased risk for pancreatic cancer as part of a familial cancer syndrome. The most important genes with variants increasing risk for pancreatic cancer include BRCA1, BRCA2, PALB2, ATM, CDKN2A, APC, MLH1, MSH2, MSH6, PMS2, PRSS1, and STK11. Recognition of members of high-risk families is important for understanding pancreatic cancer biology, for recommending risk reduction strategies and, in some cases, initiating cancer surveillance programs. Because the best methods for surveillance have not been established, the recommendation to refer at-risk patients to centers with ongoing research programs in pancreatic cancer surveillance is supported. Copyright © 2012 Wolters Kluwer Health, Inc. All rights reserved.