Hereditary pancreatic cancer: a clinical perspective.
Pancreatic cancer is an extraordinarily deadly disease and is responsible for over 220,000 deaths worldwide each year. One of the greatest risk factors for developing pancreatic cancer is a positive family history. Hereditary pancreatitis patients have a greatly elevated pancreatic cancer risk and individuals with cystic fibrosis may rarely develop this cancer, but often at very young ages. Various genetically linked cancer syndromes have been associated with pancreatic cancer in mutation-positive family members. Finally, familial pancreatic cancer-defined as families with two or more first-degree relatives who have pancreatic cancer but do not have a known cancer syndrome-is a known entity whose disease-causing mutation remains unidentified. This article describes research to date on hereditary pancreatic cancer, addresses how best clinicians should recognise hereditary forms of pancreatic cancer and explains the emotional burden of discovering a potentially lethal mutation. Many controversies and unanswered questions in hereditary pancreatic cancer remain.
University of Pittsburgh School of Medicine, Division of Gastroenterology, Hepatology and Nutrition, Medical Arts Building, 4th floor, Room 400.5, 3708 5th Ave. Pittsburgh, PA 15213, USA. greerjb@upmc.edu
Julia B
JB
Greer
Henry T
HT
Lynch
Randall E
RE
Brand
D055088:Early Detection of Cancer; D015972:Gene Expression Regulation, Neoplastic; D005817:Genetic Counseling; D020022:Genetic Predisposition to Disease; D005820:Genetic Testing; D006801:Humans; D009154:Mutation; D009386:Neoplastic Syndromes, Hereditary / Q000235:genetics*; D010190:Pancreatic Neoplasms / Q000235:genetics*; D010375:Pedigree; D012307:Risk Factors