Hereditary colorectal, gastric, and pancreatic cancer: comprehensive review

Author:

Seppälä Toni T1234ORCID,Burkhart Richard A56,Katona Bryson W7

Affiliation:

1. Faculty of Medicine and Health Technology, Tampere University , Tampere , Finland

2. Department of Gastrointestinal Surgery, Tampere University Hospital and TAYS Cancer Centre , Tampere , Finland

3. Applied Tumor Genomics Research Program, University of Helsinki , Helsinki , Finland

4. Department of Abdominal Surgery, Helsinki University Central Hospital , Helsinki , Finland

5. Department of Surgical Oncology, Johns Hopkins University , Baltimore, Maryland , USA

6. Department of Oncology, Sidney Kimmel Comprehensive Cancer Center , Baltimore, Maryland , USA

7. Division of Gastroenterology and Hepatology, Department of Medicine, Perelman School of Medicine, University of Pennsylvania , Philadelphia, Pennsylvania , USA

Abstract

Abstract Background Inheritance patterns show familial clustering of gastrointestinal cancers, and multiple germline conditions have now been identified that predispose to colorectal, gastric, and pancreatic cancers. Methods A narrative review based on recent relevant literature was conducted. Results Lynch syndrome, formerly known as hereditary non-polyposis colorectal cancer, increases the risk of several abdominal cancers, with the highest population prevalence. Familial adenomatous polyposis and some of the more infrequent polyposis syndromes have distinct characteristics affecting various organ-specific cancer risks. Hereditary gastric and pancreatic cancer syndromes include those also causing colorectal cancer, while additional genetic disorders predisposing only to upper gastrointestinal malignancies have been recognized more recently. Diagnosing and managing hereditary cancer syndromes requires multidisciplinary expertise and may be best managed in tertiary centres, with a need to consider patient preference and ensure shared decision-making. Conclusion Several germline conditions predispose to colorectal, gastric, and pancreatic cancer, which inform identification, surveillance regimens, prevention, cascade screening, counselling, and surgical management. The authors describe developments in the hereditary origin of colorectal, gastric, and pancreatic cancer with current recommendations in surveillance and surgical management.

Funder

Academy of Finland and iCAN Precision Medicine Flagship of Academy of Finland

Jane and Aatos Erkko Foundation

Sigrid Juselius Foundation

Emil Aaltonen Foundation

Cancer Society Finland

Relander Foundation

Jason and Julie Borrelli Lynch Syndrome Research Fund

Scott and Suzi Lustgarten Colon Cancer Research Fund

Smith Family Research Fund

King Family Fund for Lynch Syndrome Education, Outreach and Impact

Basser Center for BRCA

DeGregorio Family Foundation

Publisher

Oxford University Press (OUP)

Subject

General Medicine

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