Update on familial pancreatic cancer.
Approximately 5% to 10% of patients with pancreatic cancer have one or more first-degree relatives with this disease. A subset of these individuals have a hereditary form of pancreatic cancer designated by association with such hereditary disorders as familial adenomatous polyposis, hereditary nonpolyposis colorectal cancer, hereditary pancreatitis, or familial atypical multiple mole melanoma (FAMMM) syndrome. A subset of those FAMMM kindred with the CDKN2A (p16) germline mutation that expresses both pancreatic cancer and malignant melanoma may constitute a new hereditary pancreatic cancer-prone syndrome.
Lynch
H T
HT
Department of Preventive Medicine, Creighton University School of Medicine, 2500 California Plaza, Omaha, NE 68178, USA. htlynch@creighton.edu
Brand
R E
RE
Deters
C A
CA
Fusaro
R M
RM
D000230:Adenocarcinoma / Q000453:epidemiology / Q000235:genetics* / Q000628:therapy; D004416:Dysplastic Nevus Syndrome / Q000453:epidemiology / Q000235:genetics / Q000628:therapy; D005192:Family Health; D020022:Genetic Predisposition to Disease / Q000453:epidemiology / Q000209:etiology / Q000235:genetics*; D006801:Humans; D010190:Pancreatic Neoplasms / Q000453:epidemiology / Q000235:genetics* / Q000628:therapy; D014481:United States / Q000453:epidemiology