eMedicine Specialties > Gastroenterology > Systemic Disease

Familial Adenomatous Polyposis: Follow-up

Author: Mohammad Wehbi, MD, Assistant Professor of Medicine, Associate Program Director, Department of Gastroenterology, Atlanta Veterans Affairs Medical Center, Emory University School of Medicine
Coauthor(s): Nicole M Griglione, MD, Staff Physician, Department of Medicine, Emory University School of Medicine; Vincent W Yang, MD, PhD, R Bruce Logue Professor, Director, Division of Digestive Diseases, Department of Medicine, Professor of Hematology and Oncology, Winship Cancer Institute, Emory University School of Medicine; Kamil Obideen, MD, Assistant Professor of Medicine, Division of Digestive Diseases, Emory University School of Medicine; Consulting Staff, Division of Gastrointestinal Endoscopy, Atlanta Veterans Affairs Medical Center; Jae W Nam, MD, Fellow in Gastroenterology, Division of Digestive Diseases, Department of Medicine, Emory University School of Medicine; Consulting Staff, Department of Critical Care, Decatur Hospital; John M Carethers, MD, Professor of Medicine, Chief, Division of Gastroenterology, Department of Medicine, University of California at San Diego
Contributor Information and Disclosures

Updated: Jul 30, 2008

Follow-up

Complications

  • Colorectal cancer (100% in untreated patients)
  • Duodenal or periampullary adenocarcinoma (4-12%)
  • Desmoid formation (as many as 20%, typically postcolectomy)
  • Other cancers include the following:
  • Development of rectal cancer in patients with a retained rectum

Prognosis

  • Patients with untreated FAP have a median life expectancy of 42 years.
    • Life expectancy is extended greatly in those treated with colectomy.
    • Upper gastrointestinal cancers and desmoid tumors are the most common causes of death in patients who have undergone colectomy. This is why surveillance programs, especially after colectomy, are essential. Colectomy only addresses the risk of colon cancer development.
    • The cumulative probability of developing any type of a noncolorectal cancer, mostly periampullary tumors, is 11% by age 50 years and 52% by age 75 years.

Patient Education

  • Educate patients about the need for cancer surveillance after colectomy.
  • Inform family members so that they may undergo screening.

Miscellaneous

Medicolegal Pitfalls

  • Failure to adequately follow up and monitor for the development of other cancers (eg, rectal pouch, duodenum) may lead to legal problems. Surveillance programs are important.
  • Offer genetic counseling before any genetic testing is performed. The patients and their family members should be made aware of the limitations of genetic testing and the associated consequences. Genetic counseling should be performed by someone familiar with FAP and the genetic tests available.

Special Concerns

  • A gastroenterologist familiar with FAP should direct overall care.
  • A geneticist is part of the medical team involved in therapy for FAP.
  • Treatment frequently involves the input of a surgeon who is familiar with FAP.
 


More on Familial Adenomatous Polyposis

Overview: Familial Adenomatous Polyposis
Differential Diagnoses & Workup: Familial Adenomatous Polyposis
Treatment & Medication: Familial Adenomatous Polyposis
Follow-up: Familial Adenomatous Polyposis
Multimedia: Familial Adenomatous Polyposis
References
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References

  1. Nieuwenhuis MH, De Vos Tot Nederveen Cappel W, Botma A, et al. Desmoid tumors in a dutch cohort of patients with familial adenomatous polyposis. Clin Gastroenterol Hepatol. Feb 2008;6(2):215-9. [Medline].

  2. Ponti G, Losi L, Pellacani G, Rossi GB, Presutti L, Mattioli F, et al. Wnt pathway, angiogenetic and hormonal markers in sporadic and familial adenomatous polyposis-associated juvenile nasopharyngeal angiofibromas (JNA). Applied Immunohistochemistry & Molecular Morphology [serial online]. January 25, 2008;Available from: Pubmed. Accessed March 15, 2008. Available at http://www.appliedimmunohist.com/.

  3. Bianchi LK, Burke CA, Bennett AE, et al. Fundic gland polyp dysplasia is common in familial adenomatous polyposis. Clin Gastroenterol Hepatol. Feb 2008;6(2):180-5. [Medline].

  4. Friederich P, van Heumen BW, Nagtegaal ID, et al. Increased epithelial cell proliferation in the ileal pouch mucosa of patients with familial adenomatous polyposis. Virchows Arch. Sep 2007;451(3):659-67. [Medline]. [Full Text].

  5. Iaquinto G, Fornasarig M, Quaia M, et al. Capsule endoscopy is useful and safe for small-bowel surveillance in familial adenomatous polyposis. Gastrointest Endosc. Jan 2008;67(1):61-7. [Medline].

  6. Bresalier RS. Sleisenger and Fordtran's Gastrointestinal and Liver Disease. 2006;2759-2810.

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  14. Giardiello FM, Hamilton SR, Krush AJ, et al. Treatment of colonic and rectal adenomas with sulindac in familial adenomatous polyposis. N Engl J Med. May 6 1993;328(18):1313-6. [Medline].

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Further Reading

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Keywords

familial adenomatous polyposis, FAP, adenomatous polyposis syndrome, adenomatous polyps, colon cancer, colorectal cancer, adenomatous polyposis coli, colorectal carcinoma, colonic polyps, colonic neoplasia, rectal bleeding, adenomatous polyposis coli gene, APC gene, Gardner syndrome, Turcot syndrome, attenuated adenomatous polyposis coli, AAPC, desmoid tumors, intestinal polyposis, colectomy, rectal resection, duodenal adenocarcinoma, periampullary adenocarcinoma, medulloblastoma, hepatoblastoma, thyroid cancers, adrenal cancers, upper gastrointestinal cancers

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Contributor Information and Disclosures

Author

Mohammad Wehbi, MD, Assistant Professor of Medicine, Associate Program Director, Department of Gastroenterology, Atlanta Veterans Affairs Medical Center, Emory University School of Medicine
Mohammad Wehbi, MD is a member of the following medical societies: American College of Physicians, American Gastroenterological Association, and American Medical Association
Disclosure: Nothing to disclose.

Coauthor(s)

Nicole M Griglione, MD, Staff Physician, Department of Medicine, Emory University School of Medicine
Nicole M Griglione, MD is a member of the following medical societies: American Medical Association and Illinois State Medical Society
Disclosure: Nothing to disclose.

Vincent W Yang, MD, PhD, R Bruce Logue Professor, Director, Division of Digestive Diseases, Department of Medicine, Professor of Hematology and Oncology, Winship Cancer Institute, Emory University School of Medicine
Vincent W Yang, MD, PhD is a member of the following medical societies: Alpha Omega Alpha, American Gastroenterological Association, American Society for Clinical Investigation, and Association of American Physicians
Disclosure: Nothing to disclose.

Kamil Obideen, MD, Assistant Professor of Medicine, Division of Digestive Diseases, Emory University School of Medicine; Consulting Staff, Division of Gastrointestinal Endoscopy, Atlanta Veterans Affairs Medical Center
Kamil Obideen, MD is a member of the following medical societies: American College of Gastroenterology, American Gastroenterological Association, and American Society for Gastrointestinal Endoscopy
Disclosure: Nothing to disclose.

Jae W Nam, MD, Fellow in Gastroenterology, Division of Digestive Diseases, Department of Medicine, Emory University School of Medicine; Consulting Staff, Department of Critical Care, Decatur Hospital
Jae W Nam, MD is a member of the following medical societies: American College of Gastroenterology
Disclosure: Nothing to disclose.

John M Carethers, MD, Professor of Medicine, Chief, Division of Gastroenterology, Department of Medicine, University of California at San Diego
John M Carethers, MD is a member of the following medical societies: Alpha Omega Alpha, American Association for Cancer Research, American College of Gastroenterology, American College of Physicians, and American Gastroenterological Association
Disclosure: Nothing to disclose.

Medical Editor

John Gunn Lee, MD, Director of Pancreaticobiliary Service, Associate Professor, Department of Internal Medicine, Division of Gastroenterology, University of California at Irvine School of Medicine
John Gunn Lee, MD is a member of the following medical societies: American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, and American Society for Gastrointestinal Endoscopy
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Simmy Bank, MD, Chair, Professor, Department of Internal Medicine, Division of Gastroenterology, Long Island Jewish Hospital, Albert Einstein College of Medicine
Disclosure: Nothing to disclose.

CME Editor

Alex J Mechaber, MD, FACP, Assistant Dean for Medical Curriculum, Associate Professor of Medicine, Division of General Internal Medicine, University of Miami Miller School of Medicine
Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and Society of General Internal Medicine
Disclosure: Nothing to disclose.

Chief Editor

Julian Katz, MD, Clinical Professor of Medicine, Drexel University College of Medicine; Consulting Staff, Department of Medicine, Section of Gastroenterology and Hepatology, Hospital of the Medical College of Pennsylvania
Julian Katz, MD is a member of the following medical societies: American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, American Geriatrics Society, American Medical Association, American Society for Gastrointestinal Endoscopy, American Society of Law Medicine and Ethics, American Trauma Society, Association of American Medical Colleges, and Physicians for Social Responsibility
Disclosure: Nothing to disclose.

 
 
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