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Canadian Journal of Gastroenterology - Volume 18 2004, Issue 1, Pages 17-21

Original Article

Alberta Cancer Genetics Program, Walter C Mackenzie Health Sciences Centre, Edmonton, Alberta, Canada

Department of Medicine and Medical Genetics, Walter C Mackenzie Health Sciences Centre, Edmonton, Alberta, Canada

Received 9 September 2003; Accepted 7 November 2003

Copyright © 2004 Hindawi Publishing Corporation. This open-access article is distributed under the terms of the Creative Commons Attribution Non-Commercial License CC BY-NC, which permits reuse, distribution and reproduction of the article, provided that the original work is properly cited and the reuse is restricted to noncommercial purposes.


OBJECTIVE: Pancreatic cancer is known to aggregate in some families and has been associated with a wide variety of cancer syndromes. The authors describe their experience with pancreatic cancer and the range of associated cancer syndromes.

METHODS: The charts of all patients seen for concern of a hereditary cancer syndrome in the Cancer Genetics Clinic at the University of Alberta between 1995 and 2002 were reviewed.

RESULTS: Forty families reported a personal or family history of pancreatic cancer in the context of a possible hereditary cancer syndrome. Three additional families reported a history of pancreatitis. Twenty-four 56% of those families were suspected of having a hereditary breast and ovarian cancer syndrome. A further seven 16% were suspected of having hereditary nonpolyposis colon cancer. Only three 7% were believed to be at risk for a site-specific pancreatic cancer syndrome. Another three 7% were suspicious for hereditary pancreatitis. The remaining family histories were suggestive of Li-Fraumeni syndrome, von Hippel-Lindau syndrome or a nonspecific cancer predisposition.

CONCLUSIONS: With such a wide variety of hereditary cancer syndromes associated with pancreatic cancer, an accurate assessment of the family history is essential to determine the most appropriate cancer screening for at-risk family members and to guide any molecular testing that may be offered.

Autor: Margaret Lilley and Dawna Gilchrist



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