Inherited pancreatic cancer: improvements in our understanding of genetics and screening.
Inherited pancreatic cancers represent approximately 5-10% of all pancreatic cancers. Pancreatic cancer may be inherited as part of a known cancer syndrome or in association with hereditary pancreatitis or cystic fibrosis. However, most inherited pancreatic cancers do not occur in the context of a known syndrome, and these are referred to as familial pancreatic cancers. Growing evidence suggests the presence of a single autosomal dominant gene in familial pancreatic cancer kindreds, and a susceptibility locus on chromosome 4 has recently been identified in one such family. Pancreatic cancer is believed to arise from pancreatic dysplasia, and familial pancreatic cancer kindreds represent a particularly high-risk population for whom effective screening strategies are needed. One promising strategy has used endoscopic ultrasound to detected pancreatic dysplasia in members of familial pancreatic cancer kindreds.
Rulyak
Stephen J
SJ
Division of Gastroenterology, University of Washington Medical Center, Box 356424, 1959 NE Pacific St., Seattle, WA 98195, USA.
Brentnall
Teresa A
TA
D005192:Family Health; D020022:Genetic Predisposition to Disease; D006801:Humans; D009386:Neoplastic Syndromes, Hereditary; D010190:Pancreatic Neoplasms / Q000175:diagnosis / Q000209:etiology / Q000235:genetics* / Q000628:therapy; D012307:Risk Factors