Hereditary Pancreatic Cancer Syndromes

Ashton A Connor; Steven Gallinger

doi:10.1016/j.soc.2015.06.007

Hereditary Pancreatic Cancer Syndromes

Surg Oncol Clin N Am. 2015 Oct;24(4):733-64. doi: 10.1016/j.soc.2015.06.007. Epub 2015 Aug 1.

Authors

Ashton A Connor¹, Steven Gallinger²

Affiliations

¹ Division of General Surgery, Department of Surgery, Faculty of Medicine, University of Toronto, Toronto, Ontario, Canada.
² Division of General Surgery, Department of Surgery, Faculty of Medicine, University of Toronto, Toronto, Ontario, Canada. Electronic address: steven.gallinger@uhn.ca.

PMID: 26363539
DOI: 10.1016/j.soc.2015.06.007

Abstract

Despite decades of scientific and clinical research, pancreatic ductal adenocarcinoma (PDAC) remains a lethal malignancy. The clinical and pathologic features of PDAC, specifically the known environmental and genetic risk factors, are reviewed here with special emphasis on the hereditary pancreatic cancer (HPC) syndromes. For these latter conditions, strategies are described for their identification, for primary and secondary prevention in unaffected carriers, and for disease management in affected carriers. Nascent steps have been made toward personalized medicine based on the rational use of screening, tumor subtyping, and targeted therapies; these have been guided by growing knowledge of HPC syndromes in PDAC.

Keywords: Adenocarcinoma; Cancer; Exocrine; Familial; Genetics; Hereditary; Pancreas; Syndrome.

Publication types

Review

MeSH terms

Disease Management
Genetic Predisposition to Disease*
Humans
Neoplastic Syndromes, Hereditary / diagnosis
Neoplastic Syndromes, Hereditary / genetics*
Pancreatic Neoplasms / diagnosis
Pancreatic Neoplasms / genetics*
Risk Assessment