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FAP, gastric cancer, and genetic counseling featuring children and young adults: a family study and review

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Abstract

Familial adenomatous polyposis is a highly complex and multifaceted colorectal cancer prone disorder which is often significantly confounded by extracolonic cancers inclusive of gastric cancer, a significant problem in the Orient. Gastric cancer in familial adenomatous polyposis is heavily influenced by fundic gland polyps which are often so voluminous as to defy effective endoscopic surveillance. This study involves more than two decades of investigation of an attenuated familial adenomatous polyposis family where gastric cancer posed an early diagnostic problem because it was obscured by multiple fundic gland polyps. Fundic gland polyps are common in familial adenomatous polyposis and attenuated familial adenomatous polyposis and, if voluminous, may interfere with effective endoscopic gastric cancer surveillance. This family is believed to be the first of its type reported with focus upon education and genetic counseling in the setting of a family information service. Cancer control in familial adenomatous polyposis may be partially resolved through use of familial colorectal cancer registries, with greater attention to family history and its interpretation, genetic counseling, and clinical translation for diagnosis and management.

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Acknowledgments

Financial support

This paper was supported by revenue from Nebraska cigarette taxes awarded to Creighton University by the Nebraska Department of Health and Human Services. Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the State of Nebraska or the Nebraska Department of Health and Human Services. Support was also given by the National Institutes of Health through grant #1U01 CA 86389. Dr. Henry Lynch’s work is partially funded through the Charles F. and Mary C. Heider Chair in Cancer Research, which he holds at Creighton University.

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Author notes

  1. Victoria Graeve

    Present address: Niedfelt Nursing Research Center, University of Nebraska Medical Center, Omaha, NE, USA

Authors and Affiliations

  1. Department of Preventive Medicine and Public Health, Creighton University School of Medicine, 2500 California Plaza, Omaha, NE, 68178, USA

    Henry T. Lynch, Carrie Snyder, Jane Lynch & Victoria Graeve

  2. GI Oncology, University of North Carolina at Chapel Hill, Chapel Hill, NC, USA

    Janine M. Davies

  3. Internal Medicine Department/Gastroenterology, Creighton University School of Medicine, Omaha, NE, USA

    Stephen Lanspa

  4. Department of Pathology, Creighton University Medical Center, Omaha, NE, USA

    Zoran Gatalica

  5. Department of Surgery/Surgical Oncology, Creighton University Medical Center, Omaha, NE, USA

    Jason Foster

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  1. Henry T. Lynch
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  2. Carrie Snyder
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  3. Janine M. Davies
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  5. Jane Lynch
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Correspondence to Henry T. Lynch.

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Lynch, H.T., Snyder, C., Davies, J.M. et al. FAP, gastric cancer, and genetic counseling featuring children and young adults: a family study and review. Familial Cancer 9, 581–588 (2010). https://doi.org/10.1007/s10689-010-9352-7

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  • DOI: https://doi.org/10.1007/s10689-010-9352-7

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