Familial pancreatic cancer.

Journal Article (Journal Article;Review)

CONTEXT: Approximately 5% to 10% of individuals with pancreatic cancer report a history of pancreatic cancer in a close family member. In addition, several known genetic syndromes, such as familial breast cancer (BRCA2), the Peutz-Jeghers syndrome, and the familial atypical multiple mole melanoma syndrome, have been shown to be associated with an increased risk of pancreatic cancer. The known genes associated with these conditions can explain only a portion of the clustering of pancreatic cancer in families, and research to identify additional susceptibility genes is ongoing. OBJECTIVE: To provide an understanding of familial pancreatic cancer and the pathology of familial exocrine pancreatic cancers. DATA SOURCES: Published literature on familial aggregation of pancreatic cancer and familial exocrine pancreatic tumors. CONCLUSIONS: Even in the absence of predictive genetic testing, the collection of a careful, detailed family history is an important step in the management of all patients with pancreatic cancer. While most pancreatic cancers that arise in patients with a family history are ductal adenocarcinomas, certain subtypes of pancreatic cancer have been associated with familial syndromes. Therefore, the histologic appearance of the pancreatic cancer itself, and/or the presence and appearance of precancerous changes in the pancreas, may increase the clinical index of suspicion for a genetic syndrome.

Full Text

Duke Authors

Cited Authors

  • Shi, C; Hruban, RH; Klein, AP

Published Date

  • March 2009

Published In

Volume / Issue

  • 133 / 3

Start / End Page

  • 365 - 374

PubMed ID

  • 19260742

Pubmed Central ID

  • PMC8281570

Electronic International Standard Serial Number (EISSN)

  • 1543-2165

Digital Object Identifier (DOI)

  • 10.5858/133.3.365


  • eng

Conference Location

  • United States