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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.

Your Question

What is the recommended follow up or management of AFAP after the colon has been removed? The small intestine is connected to the rectum. I have had polyps in the rectum and also in the stomach since the colon was removed. How often do I need to do follow up tests to make sure nothing is growing in there?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

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.[1] 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.[2] 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.[2]

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.[1]

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

What is the recommended follow-up for attenuated familial adenomatous polyposis after the colon has been removed?

There is limited literature available that is specific to attenuated familial adenomatous polyposis (AFAP). Therefore, many experts suggest that people with AFAP follow the same screening guidelines as those with classic familial adenomatous polyposis (FAP), which may include:[2]
  • If total colectomy with ileo-anal pull-through was performed: routine screening of the ileal pouch every two years
  • If subtotal colectomy (removal of part of the colon) was performed: screening of the remaining rectum every six to 12 months, depending on the number of polyps that develop. Cancer may still occur in the remaining rectum, but the risk is much lower.
  • Annual physical examination with palpation of the thyroid with consideration of follow-up ultrasound examination and fine-needle aspiration if thyroid nodules are present
Extra colonic manifestations of FAP generally require affected people to follow a lifelong screening program for the diagnosis and treatment of desmoid tumors and benign and malignant tumors of the duodenum (a section of the small intestine), stomach, bones, skin, and other tissues.[4]

Additional and more detailed information about follow-up recommendations for individuals with FAP or AFAP, including post-surgery recommendations is available at the following links:
Last updated: 11/28/2014

References
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.