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Changing causes of mortality in patients with familial adenomatous polyposis

Abstract

Widespread use of prophylactic colectomy has resulted in a reduction in the incidence of colorectal cancer in familial adenomatous polyposis (FAP) patients. A retrospective chart review of families registered at the Steve Atanas Stavro Familial Gastrointestinal Cancer Registry in Toronto was performed to determine whether the decrease in the number of patients developing colorectal cancer implies that causes of mortality in FAP patients are shifting to that of extracolonic manifestations of FAP. Information was available on 140 deaths within 158 families and among 461 individuals with FAP. When stratified by decade, from the 1930s to the 1990s, the ratio of deaths caused by extracolonic manifestations of FAP compared with deaths caused by colorectal cancer was noted to have risen. Even though most deaths in FAP patients are still from colorectal cancer, it appears that screening policies and prophylactic colectomy have resulted in a reduction in the number of FAP patients who develop colorectal cancer. Thus, in recent decades, a greater percentage of deaths in FAP patients appears to be attributable to extracolonic manifestations of the disease.

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References

  1. 1.

    Spigelman AD. Familial adenomatous polyposis: recent genetic advances. Br J Surg 1994;81:321–2.

    Google Scholar 

  2. 2.

    Iwama T, Mishima Y, Utsunomiya J. The impact of familial adenomatous polyposis on the tumorigenesis and mortality at the several organs. Ann Surg 1993;217:101–8.

    Google Scholar 

  3. 3.

    Jagelman DG. Extracolonic manifestations of familial adenomatous polyposis. Semin Surg Oncol 1987;3:88–91.

    Google Scholar 

  4. 4.

    Bulow S. Incidence of associated diseases in familial polyposis coli. Semin Surg Oncol 1987;3:84–7.

    Google Scholar 

  5. 5.

    Jagelman DG. Extra-colonic manifestations of familial adenomatous polyposis. Oncology 1991;5:23–7.

    Google Scholar 

  6. 6.

    Berk T, Stern HS. Screening, management, and surveillance for families with familial adenomatous polyposis. Gastrointest Endosc Clin N Am 1993;3:725–36.

    Google Scholar 

  7. 7.

    Arvanitis ML, Jagelman DG, Fazio VW, Lavery IC, McGannon E. Mortality in patients with familial adenomatous polyposis. Dis Colon Rectum 1990;33:639–42.

    Google Scholar 

  8. 8.

    Bertario L, Presciuttini S, Sala P, Rossetti C, Pietroiusti M. Causes of death and post surgical survival in familial adenomatous polyposis: results from the Italian Registry. Semin Surg Oncol 1994;10:225–34.

    Google Scholar 

  9. 9.

    Groden J, Thliveris A, Samowitz W,et al. Identification and characterization of the familial adenomatous polyposis coli gene. Cell 1991;66:589–600.

    Google Scholar 

  10. 10.

    Bapat B, Berk T, Mitri A, Cohen Z, Gallinger S, Stern H. Characterization of two novel adenomatous polyposis coli (APC) gene mutations in patients with familial adenomatous polyposis. Hum Mutat 1994;4:255–6.

    Google Scholar 

  11. 11.

    Rustgi AK. Hereditary gastrointestinal polyposis and nonpolyposis syndromes. N Engl J Med 1994;331:1694–702.

    Google Scholar 

  12. 12.

    Penna C, Phillips RK, Tiret E, Spigelman AD. Surgical polypectomy of duodenal adenomas in familial adenomatous polyposis: experience of two European centres. Br J Surg 1993;80:1027–9.

    Google Scholar 

  13. 13.

    Iwama T, Tomita H, Kawachi Y,et al. Indications for local excision of ampullary lesions associated with familial adenomatous polyposis. J Am Coll Surg 1994;179:462–4.

    Google Scholar 

  14. 14.

    Giardiello FM, Krush AJ, Petersen GM,et al. Phenotypic variability of familial adenomatous polyposis in 11 unrelated families with identical APC gene mutation. Gastroenterology 1994;106:1542–7.

    Google Scholar 

  15. 15.

    Paul P, Letteboer T, Gelbert L, Groden J, White R, Coppes MJ. Identical APC exon 15 mutations result in a variable phenotype in familial adenomatous polyposis. Hum Mol Genet 1993;2:925–31.

    Google Scholar 

  16. 16.

    Gurbuz AK, Giardiello FM, Petersen GM,et al. Desmoid tumors in familial adenomatous polyposis. Gut 1994;35:377–81.

    Google Scholar 

  17. 17.

    Sanabria JR, Croxford R, Berk TB, Cohen Z, Bapat BV, Gallinger S. Familial segregation in the occurrence and severity of periampullary neoplasms in familial adenomatous polyposis. Am J Surg 1996;171:136–41.

    Google Scholar 

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Correspondence to Steven Gallinger M.D..

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Read at the meeting of The American Society of Colon and Rectal Surgeons, Montreal, Quebec, Canada, May 7 to 12, 1995, and at the Clinical Research Society of Toronto, Toronto, Ontario, Canada, April 29, 1995.

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Belchetz, L.A., Berk, T., Bapat, B.V. et al. Changing causes of mortality in patients with familial adenomatous polyposis. Dis Colon Rectum 39, 384–387 (1996). https://doi.org/10.1007/BF02054051

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Key words

  • Familial adenomatous polyposis
  • Colorectal carcinoma
  • Desmoid tumors
  • Periampullary tumors