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Genetic and Rare Diseases Information Center (GARD)

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Attenuated familial adenomatous polyposis


Other Names for this Disease

  • AAPC
  • AFAP
  • Attenuated adenomatous polyposis coli
  • Attenuated familial polyposis coli
  • Attenuated FAP
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Overview

What is attenuated familial adenomatous polyposis?

How might attenuated familial adenomatous polyposis be treated?

What is attenuated familial adenomatous polyposis?

Attenuated familial adenomatous polyposis (AFAP) is an inherited condition that causes cancer of the large intestine (colon) and rectum. It is a milder form of classic familial adenomatous polyposis that is characterized by fewer colon polyps (an average of 30) and a delay in the development of colon cancer (average age 50 to 55 years). Other signs and symptoms may include dental abnormalities; desmoid tumors; and benign and malignant (cancerous) tumors of the duodenum (a section of the small intestine), stomach, bones, skin, thyroid and other tissues. AFAP is caused by mutations in the APC gene and is inherited in an autosomal dominant manner. AFAP is generally managed with regular screening to detect if and when polyps develop.[1]
Last updated: 11/28/2014

How might attenuated familial adenomatous polyposis be treated?

Attenuated familial adenomatous polyposis (AFAP) is generally managed with regular screening to detect if and when polyps develop.[2] Screening by colonoscopy has been recommended for affected people starting at age 20 to 25 years. People with polyps may undergo polypectomy (removal of polyps) followed by continued screenings every one to three years, depending on the number of polyps.[1] A prophylactic colectomy (removal of all or part of the colon) may be considered in people with too many adenomas to remove or those who cannot undergo screening.[3] About one third of people with AFAP have few enough colon polyps that screening with periodic polypectomy is sufficient.[1]

Because individuals with AFAP can also develop duodenal adenomas and other cancers, upper endoscopy is typically recommended starting at age 20 to 30 years and then every one to three years, depending on the number of polyps. There is currently no consensus on screening for tumors that occur outside of the colon, so it has been suggested that affected individuals are managed as if they have classic FAP.[3]

A number of drugs such as celecoxib and sulindac reportedly have been successful at reducing the number and the size of polyps in affected people, but these drugs generally help to prevent further complications and are not considered adequate treatment.[2]

Additional and more detailed information about the treatment and management of FAP, including AFAP, is available on eMedicine's Web site and can be viewed by clicking here.
Last updated: 11/28/2014

References
  1. Kory W Jasperson, MS and Randall W Burt, MD. APC-Associated Polyposis Conditions. GeneReviews. March 27, 2014; http://www.ncbi.nlm.nih.gov/books/NBK1345/.
  2. Mohammad Wehbi. Familial Adenomatous Polyposis. eMedicine. September 1, 2011; http://emedicine.medscape.com/article/175377-overview. Accessed 12/16/2011.
  3. Peter A L Bonis, MD; Dennis J Ahnen, MD; Lisen Axell, MS, CGC. Familial adenomatous polyposis and MUTYH associated polyposis: Screening and management of patients and families. UpToDate. April 2014;


Other Names for this Disease
  • AAPC
  • AFAP
  • Attenuated adenomatous polyposis coli
  • Attenuated familial polyposis coli
  • Attenuated FAP
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.