Skip to main content

Advertisement

Log in

Gastric cancer in FAP: a concerning rise in incidence

  • Short Communication
  • Published:
Familial Cancer Aims and scope Submit manuscript

Abstract

The highest cancer risks in familial adenomatous polyposis (FAP) include colorectal, duodenal, and thyroid for which surveillance is recommended. Nearly all patients with FAP have gastric fundic gland polyposis (FGP), but gastric cancers are rarely reported with a similar incidence as the general population. We describe a recent, sudden increase in the incidence of gastric cancer in FAP. Seven of the ten cases were diagnosed in the last 20 months. Comparing our population to the SEER database for gastric cancer, the standardized incidence ratio is 140. All cases arose in patients with a carpeting of FGP and associated with large mounds of proximal gastric polyps. Nearly all patients were under upper endoscopic surveillance. This is a concerning observation and reflects a change in the phenotypic presentation of FAP in Western patients.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

References

  1. Spigelman AD, Williams CB, Talbot IC et al (1989) Upper gastrointestinal cancer in patients with familial adenomatous polyposis. Lancet 2(8666):783–785

    Article  CAS  PubMed  Google Scholar 

  2. Jarrar AM, Milas M, Mitchell J et al (2011) Screening for thyroid cancer in patients with familial adenomatous polyposis. Ann Surg 252:515–521

    Article  Google Scholar 

  3. Syngal S, Brand RE, Church JM et al (2015) ACG clinical guideline: genetic testing and management of hereditary gastrointestinal cancer syndromes. Am J Gastroenterol 110:223–262

    Article  PubMed  PubMed Central  Google Scholar 

  4. National Comprehensive Cancer Network (2015) NCCN Clinical Practice Guidelines in Oncology: Genetic/Familial High-Risk Assessment: Colorectal. Version 2.2015. Fort Washington, PA: National Comprehensive Cancer Network

    Google Scholar 

  5. Vasen HFA, Moslen G, Alonso A et al (2008) Guidelines for the clinical management of familial adenomatous polyposis (FAP). Gut 57:704–713

    Article  CAS  PubMed  Google Scholar 

  6. Jagelman DG, DeCosse JJ, Bussey HJ (1988) Upper gastrointestinal cancer in familial adenomatous polyposis. Lancet 1:1149–1150

    Article  CAS  PubMed  Google Scholar 

  7. Bianchi LK, Burke CA, Bennett AE et al (2008) Fundic gland polyp dysplasia is common in familial adenomatous polyposis. Clin Gastroenterol Hepatol 6:180–185

    Article  PubMed  Google Scholar 

  8. Wood LD, Salaria SN, Cruise MW, Giardiello FM, Montgomery EA (2014) Upper GI tract lesions in familial adenomatous polyposis (FAP): enrichment of pyloric gland adenomas and other gastric and duodenal neoplasms. Am J Surg Pathol 38(3):389–393

    Article  PubMed  PubMed Central  Google Scholar 

  9. Matsubara A, Sekine S, Kushima R et al (2013) Frequent GNAS and KRAS mutations in pyloric gland adenoma of the stomach and duodenum. J Pathol 229(4):579–587

    Article  CAS  PubMed  Google Scholar 

  10. Li J, Woods SL, Healey S et al (2016) Point mutations in Exon 1B of APC reveal gastric adenocarcinoma and proximal polyposis of the stomach as a familial adenomatous polyposis variant. Am J Hum Genet 98:830–842

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  11. Bass AJ, Thorsson V, Shmulevich I, et al (2014) Comprehensive molecular characterization of gastric adenocarcinoma. Nature 513(7517):202–209

    Article  PubMed Central  Google Scholar 

  12. Abraham SC, Nobukawa B, Giardiello FM, Hamilton SR, Wu TT (2000) Fundic gland polyps in familial adenomatous polyposis: neoplasms with frequent somatic APC gene alterations. Am J Pathol 157:747–754

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  13. Abraham SC, Nobukawa B, Giardiello FM, Hamilton SR, Wu TT (2001) Sporadic fundic gland polyps: common gastric polyps arising through activating mutations in the Β-catenin gene. Am J Pathol 158:1005–1010

    Article  CAS  PubMed  PubMed Central  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Gautam Mankaney.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Mankaney, G., Leone, P., Cruise, M. et al. Gastric cancer in FAP: a concerning rise in incidence. Familial Cancer 16, 371–376 (2017). https://doi.org/10.1007/s10689-017-9971-3

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10689-017-9971-3

Keywords

Navigation