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International Journal of Surgical Oncology
Volume 2011 (2011), Article ID 154673, 10 pages
Review Article

Hereditary Pancreatic and Hepatobiliary Cancers

1Department of Surgery, Saint Agnes Hospital, Baltimore, MD 21229, USA
2Department of Surgery, The Johns Hopkins Hospital, Baltimore, MD 21231, USA

Received 4 April 2011; Accepted 28 April 2011

Academic Editor: Benedito Mauro Rossi

Copyright © 2011 Ashraf Haddad et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Hereditary etiologies of pancreatic and hepatobiliary cancers are increasingly recognized. An estimated >10% of pancreatic and increasing number of hepatobiliary cancers are hereditary. The cumulative risk of hereditary pancreatic cancer ranges from measurable but negligible in cystic fibrosis to a sobering 70% in cases of hereditary pancreatitis. Candidates for pancreatic cancer surveillance are those with a risk pancreatic cancer estimated to be >10-fold that of the normal population. Screening for pancreatic cancer in high-risk individuals is typically performed by endoscopic ultrasound and should begin at least 10 years prior to the age of the youngest affected relative. Disease states known to be associated with increased risk of hepatocellular cancer include hereditary hemochromatosis, autoimmune hepatitis, porphyria, and α1-antitrypsin deficiency, with relative risks as high as 36-fold. Although much less is known about hereditary bile-duct cancers, Muir-Torre syndrome and bile salt export pump deficiency are diseases whose association with hereditary carcinogenesis is under investigation.