Skip to main content
SpringerLink
Log in

Slow progression of periampullary neoplasia in familial adenomatous polyposis

  • Published:
Journal of Gastrointestinal Surgery

Abstract

Variable endoscopic surveillance protocols and treatment strategies have been proposed for periampullary neoplasia in familial adenomatous polyposis (FAP), primarily because of the lack of long-term, prospective natural history data. A total of 115 patients with FAP were followed prospectively for 10 years with periodic side-viewing upper gastrointestinal endoscopy by a single surgeon. The appearance of the duodenum was classified as stages 1 to 5. Statistical analysis included one-way analysis of variance for age comparisons between stage groupings and Kaplan-Meier analysis for the lifetime risks of having a particular stage of duodenal polyposis. Eighty-seven patients had multiple endoscopies over an average of 6.6 years. Thirty-three subjects had a change in stage, within an average time of 3.9 years at an average age of 41 years. The risk of having stage 3 or 4 duodenal neoplasia increased exponentially after the age of 40. The degree of dysplasia did not correlate with stage at initial classification. Progression of neoplasia in the duodenum of patients with FAP is slow. The severity of duodenal polyposis increases with age and is not influenced by the initial stage. The average time for progression of adenoma to carcinoma is likely long.

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

Access this article

Log in via an institution

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

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Griffioen G, Bus PJ, Vasen HFA, Verspaget HW, Lamers CB. Extracolonic manifestations of familial adenomatous polyposis: Desmoid tumors, and upper gastrointestinal adenomas and carcinomas. Scand J Gastroenterol 1998;33 225(Suppl):85–91.

    Article  CAS  Google Scholar 

  2. Offerhaus GJA, Entlus MM, Giardiello FM. Upper gastrointestinal polyps in familial adenomatous polyposis. Hepatogastroenterology 1999;46:667–669.

    CAS  PubMed  Google Scholar 

  3. Spigelman AD. Screening modalities in familial adenomatous polyposis and hereditary nonpolyposis colorectal cancer. Gastrointest Endosc Clin North Am 1997;81/2-86.

    Google Scholar 

  4. Belchetz LA, Berk T, Bapat BV, Cohen Z, Gallinger S. Changing causes of mortality in patients with familial adenomatous polyposis. Dis Colon Rectum 1996;39:384–387.

    Article  CAS  PubMed  Google Scholar 

  5. Galle TS, Juel K, Bulow S. Causes of death in familial adenomatous polyposis. Scand J Gastroenterol 1999;34:808–812.

    Article  CAS  PubMed  Google Scholar 

  6. Wallace MH, Phillips RK. Upper gastrointestinal disease in patients with familial adenomatous polyposis. Br J Surg 1998;85:742–750.

    Article  CAS  PubMed  Google Scholar 

  7. Norton ID, Gostout CJ. Management of periampullary adenomas. Dig Dis 1998;16:266–273.

    Article  CAS  PubMed  Google Scholar 

  8. Sanabria JR, Croxford R, Berk T, Cohen Z, Bapat BV, Gallinger S. Familial segregation in the occurrence and severity of periampullary neoplasms in familial adenomatous polyposis. Am J Surg 1996;171:136–141.

    Article  CAS  PubMed  Google Scholar 

  9. Nugent KP, Spigelman AD, Phillips RKS. Risk of extracolonic cancer in familial adenomatous polyposis. Br J Surg 1996;83:1121–1122.

    Article  CAS  PubMed  Google Scholar 

  10. Jagleman DG, DeCosse JJ, Bussey HJR, and The Leeds Castle Polyposis Group. Upper gastrointestinal cancer in familial adenomatous polyposis. Lancet 1988;8595:1149–1150.

    Article  Google Scholar 

  11. Nugent KP, Spigelman AD, Williams CB, Talbot IC, Phillips KS. Surveillance of duodenal polyps in familial adenomatous polyposis: Progress report. J R Soc Med 1994;87:704–706.

    CAS  PubMed  Google Scholar 

  12. Spigelman AD, Talbot IC, Penna C, Nugent KP, Phillips RK, Costello C, DeCosse JJ. Evidence for adenoma-carcinoma sequence in the duodenum of patients with familial adenomatous polyposis. J Clin Pathol 1994;47:709–710.

    Article  CAS  PubMed  Google Scholar 

  13. Kashiwagi H, Spigelman AD. Gastroduodenal lesions in familial adenomatous polyposis. Surg Today 2000;30:675–682.

    Article  CAS  PubMed  Google Scholar 

  14. Goedde TA, Rodriguez-Bigas MA, Herrera L, Petrelli NJ. Gastroduodenal polyps in familial adenomatous polyposis. Surg Oncol 1992;1:357–361.

    Article  CAS  PubMed  Google Scholar 

  15. Debinski HS, Spigelman AD, Hatfield A, Williams CB, Phillips RKS. Upper intestinal surveillance in familial adenomatous polyposis. Eur J Cancer 1995;31:1149–1153.

    Article  Google Scholar 

  16. Bulow S, Alm T, Fausa O, Hultcrantz R, Jarvinen H, Vasen H, the DAF Project Group. Duodenal adenomatosis in familial adenomatous polyposis. Int J Colorectal Dis 1995;10:43–46.

    Article  CAS  PubMed  Google Scholar 

  17. Sawada T, Muto T. Role of upper gastrointestinal surveillance in patients with familial adenomatous polyposis. Gastrointest Endosc Clin North Am 1997;7:99–110.

    CAS  Google Scholar 

  18. Björk J, Akerbrant H, Iselius L, Bergman A, Engwall Y, Wahlstrom J, Martinsson T, Nordling M, Hultcrantz R. Periampullary adenomas and adenocarcinomas in familial adenomatous polyposis: Cumulative risks and APC gene mutations. Gastroentelology 2001;121:1127–1135.

    Article  Google Scholar 

  19. Matsumoto T, Iida M, Nakamura S, Hizawa K, Yao T, Tsuneyoshi M, Fujishima M. Natural history of ampullary adenoma in familial adenomatous polyposis: Reconfirmation of benign nature during extended surveillance. Am J Gastroenterol 2000;95:1557–1562.

    Article  CAS  PubMed  Google Scholar 

  20. Burke CA, Beck GJ, Church JM, van Stolk RU. The natural history of untreated duodenal and ampullary adenomas in patients with familial adenomatous polyposis followed in an endoscopic surveillance program. Gastrointest Endosc 1999; 49:358–364.

    Article  CAS  PubMed  Google Scholar 

  21. Bertoni G, Sassatelli R, Nigrisoli E, Pennazio M, Tansini P, Arrigoni A, Ponz de Leon M, Rossini FP, Bedogni G. High prevalence of adenomas and microadenomas of the duodenal papilla and periampullary region in patients with familial adenomatous polyposis. Eur J Gastroenterol Hepatol 1996;8:1201–1206.

    Article  CAS  PubMed  Google Scholar 

  22. Groves CJ, Saunders BP, Spigelman AD, Phillips RSK, Duodenal cancer in patients with familial adenomatous polyposis (FAP): Results of a 10 year prospective study. Gut 2002;50:636–641.

    Article  CAS  PubMed  Google Scholar 

  23. Penna C, Phillips RKS, Tiret E, Spigelman AD. Surgical polypectomy of duodenal adenomas in familial adenomatous polyposis: Experience of two European centres. Br J Surg 1993;80:1027–1029.

    Article  CAS  PubMed  Google Scholar 

  24. Heiskanan I, Kellokumpu I, Jarvinen H. Management of duodenal adenomas in 98 patients with familial adenomatous polyposis. Endoscopy 1999;31:412–416.

    Article  Google Scholar 

  25. Kadmon M, Tandara A, Herfarth C. Duodenal adenomatosis in familial adenomatous polyposis coli. A review of the literature and results from the Heidelberg Register. Int J Colorect Dis 2001;16:63–75.

    Article  CAS  Google Scholar 

  26. Soravia C, Berk T, Haber G, Cohen Z, Gallinger S. Management of advanced duodenal polyposis in familial adenomatous polyposis. J G ASTROINTEST SURG 1997;1:474–478.

    CAS  Google Scholar 

  27. Richard CS, Berk T, Bapat BV, Haber G, Cohen Z, Gallinger S. Sulindac for periampullary polyps in FAP patients. Int J Colorect Dis 1997;12:14–18.

    Article  CAS  Google Scholar 

  28. Oshima M, Dinchuk JE, Kargman SL, Oshima H, Hancock B, Kwong E, Trzaskos JM, Evans JF, Taketo MM. Suppression of intestinal polyposis in APC knockout mice by inhibition of cylooxygenase2 (COX-2). Cell 1996;87:803–809.

    Article  CAS  PubMed  Google Scholar 

  29. Steinbach G, Lynch PM, Phillips RK, Wallace MH, Hawk E, Gordon GB, Wakabayashi N, Saunders B, Shen Y, Fujimura T, Su LK, Levin B. The effect of celecoxib, a cyclooxygenase-2 inhibitor, in familial adenomatous polyposis. N Engl J Med 2000;342:1946–1952.

    Article  CAS  PubMed  Google Scholar 

  30. Phillips RKS, Wallace MH, Lynch P, Hawk E, Gordon GB, Saunders B, Wakabayashi N, Shen Y, Zimmerman S, Godio L, Rodrigues-Bigas M, Li-Kuo Su, Sherman J, Kelloff G, Levin B, Steinbach G, The FAP Study Group. A randomised double blind placebo controlled study of celecoxib, a selective cyclooxygenase-2 inhibitor, on duodenal polyposis in familial adenomatous polyposis. Gut 2002;50:857–860.

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Surgery, Mount Sinai Hospital, Toronto, Ontario, Canada

    Kouros L. Moozar M.D. & Steven Gallinger M.D.

  2. Familial Gastrointestinal Cancer Registry, Mount Sinai Hospital, Toronto, Ontario, Canada

    Lisa Madlensky M.Sc. & Terri Berk M.S.S.A.

Authors
  1. Kouros L. Moozar M.D.
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Lisa Madlensky M.Sc.
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Terri Berk M.S.S.A.
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Steven Gallinger M.D.
    View author publications

    You can also search for this author in PubMed Google Scholar

Additional information

Supported in part by the National Cancer Institute of Canada (NCIC) and the American Society of Colon and Rectal Surgeons (ASCRS).

Rights and permissions

Reprints and permissions

About this article

Cite this article

Moozar, K.L., Madlensky, L., Berk, T. et al. Slow progression of periampullary neoplasia in familial adenomatous polyposis. J Gastrointest Surg 6, 831–837 (2002). https://doi.org/10.1016/S1091-255X(02)00062-8

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1016/S1091-255X(02)00062-8

Key words

Search

Navigation