Medication Summary

Nonsteroidal anti-inflammatory drugs (NSAIDs), including aspirin, have been consistently associated with diminished risk of colorectal cancer. Sulindac has been reported to cause regression of adenomas in patients with Gardner syndrome. NSAIDs suppress cyclooxygenase-2 (COX-2), which affects epithelial proliferation and apoptosis.

Studies by Watanabe et al suggest an important role of antagonistic agents for the prostaglandin EP1 receptor for chemoprotection against the development of colon cancer.^[47]

Many future therapies will target the actual signaling pathways disrupted by the affected genes in polyposis syndromes. For example, a trial studying rapamycin use in Cowden syndrome has been conducted.^[27]

In additional to nutritional support, steroids have also been shown to be the mainstay of medical treatment that can lead to remission in Cronkhite-Canada syndrome. Azathioprine is also used as a steroid-sparing agent.^[56]

Class Summary

Growing evidence suggests a protective role for NSAIDs against the development of colorectal cancer. In addition, a significant effect in reversing adenoma growth has been illustrated with the use of sulindac and celecoxib in patients with FAP. Aspirin may also be useful to reduce the recurrence of polyps or cancer, but because of the potential for these drugs to cause damage to the upper gastrointestinal tract, they are not routinely recommended for this purpose.

Studies have shown that APC inactivation and EGFR signaling increase COX2 expression, which may lead to intestinal neoplasia. The mechanism of NSAID-induced polyp regression is not completely known, but it thought that it is at least in part due to inhibition of cyclooxygenase 2 (COX2) and the resultant decrease in prostaglandin synthesis although a non-COX mechanism may also contribute.

The mechanism of NSAID-induced polyp regression is not known, but it thought that it is at least in part due to inhibition of cyclooxygenase 2 (COX2) and the resultant decrease in prostaglandin synthesis although non-COX mechanism may also contribute.

Sulindac (Clinoril)

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Has been reported to cause regression of adenomas in patients with Gardner syndrome (ie, FAP).

A recent study randomized patents to Sulindac 150 mg twice daily along with Erlotinib 75 mg daily versus placebo for 6 months and measured polyp burden. The patients on medications had a lower duodenal polyp burden at 6 months but many patients developed adverse events such as an acne-like rash, and it is unknown whether the medications prevent new duodenal adenomas from forming.

Class Summary

These agents inhibit COX-2, thus suppress production of prostaglandin E2 at inflammation sites.

Celecoxib (Celebrex)

View full drug information

Recently was approved by the FDA for treatment of Gardner syndrome as an adjunct to endoscopy and surgery. The mean reduction in the number of polyps was 28% with 400 mg PO bid and 12% with 100 mg PO bid (5% placebo).

Follow-up

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Media Gallery

Familial adenomatous polyposis, total colectomy specimen. The colonic mucosa is studded with innumerable sessile and small pedunculated polyps, which involve the entire length of the specimen.
Pedunculated tubular adenoma. Note the contrast between the goblet cell-rich glands along the pedicle of the polyp and in the underlying normal colonic mucosa at the bottom and the dysplastic glands in the polyp proper. The dysplastic glands are more crowded and exhibit decreased mucin production. (Hematoxylin and eosin stain; 1X magnification).
Sessile tubular adenoma. The glands on the superficial aspect of the specimen are dysplastic and exhibit increased nuclear size, hyperchromasia, crowding, and decreased mucin production. (Hematoxylin and eosin stain, 4X magnification).
Hamartomatous (Peutz-Jeghers) polyp, small bowel. This pedunculated polyp has a cerebriform appearance due to the arborizing frond-like growth with delicate finger-like projections of the stroma. (Hematoxylin and eosin stain, 1X magnification).
Hamartomatous (Peutz-Jeghers) polyp, small bowel. Closer view of the fingerlike projections of the stroma demonstrates prominent smooth muscle fascicles between the glandular elements. (Hematoxylin and eosin stain, 4X magnification).
Hamartomatous polyp, as seen in the stomach. (Endoscopic image).
Colon in familial adenomatous polyposis (FAP). (Endoscopic image).
Small bowel polyp. (Video capsule image).
Multiple large polyps in the colon. The polyp in the center of the image is situated on a stalk. (Endoscopic image).

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Author

Amit A Shah, MD Fellow, Division of Gastroenterology, Hepatology, and Nutrition, Children's Hospital of Philadelphia

Amit A Shah, MD is a member of the following medical societies: American Gastroenterological Association, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition

Disclosure: Nothing to disclose.

Coauthor(s)

Petar Mamula, MD Professor, Department of Pediatrics, Division of Gastroenterology and Nutrition, The Children's Hospital of Philadelphia, University of Pennsylvania Perelman School of Medicine

Petar Mamula, MD is a member of the following medical societies: American Academy of Pediatrics, American Gastroenterological Association, American Society for Gastrointestinal Endoscopy, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition

Disclosure: Nothing to disclose.

Eduardo D Ruchelli, MD Associate Professor of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine; Attending Physician, Department of Pathology, Children’s Hospital of Philadelphia

Eduardo D Ruchelli, MD is a member of the following medical societies: United States and Canadian Academy of Pathology, Society for Pediatric Pathology

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Stefano Guandalini, MD, AGAF Founder and Director Emeritus, Celiac Disease Center, University of Chicago; Former Chief, Section of Pediatric Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, University of Chicago Medicine; Professor Emeritus, The University of Chicago Pritzker School of Medicine

Stefano Guandalini, MD, AGAF is a member of the following medical societies: American Gastroenterological Association, European Society for Paediatric Gastroenterology, Hepatology and Nutrition, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition, North American Society for the Study of Celiac Disease

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Imaware.health.

Chief Editor

Carmen Cuffari, MD Associate Professor, Department of Pediatrics, Division of Gastroenterology/Nutrition, Johns Hopkins University School of Medicine

Carmen Cuffari, MD is a member of the following medical societies: American College of Gastroenterology, American Gastroenterological Association, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition, Royal College of Physicians and Surgeons of Canada

Disclosure: Received honoraria from Prometheus Laboratories for speaking and teaching; Received honoraria from Abbott Nutritionals for speaking and teaching. for: Abbott Nutritional, Abbvie, speakers' bureau.

Additional Contributors

Jayant Deodhar, MD Associate Professor in Pediatrics, BJ Medical College, India; Honorary Consultant, Departments of Pediatrics and Neonatology, King Edward Memorial Hospital, India

Disclosure: Nothing to disclose.

Evelyn K Hsu, MD Assistant Professor of Pediatrics, Division of Gastroenterology and Hepatology, Seattle Children's Hospital

Evelyn K Hsu, MD is a member of the following medical societies: American Association for the Study of Liver Diseases, American Gastroenterological Association, American Society of Transplantation, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition, International Pediatric Transplant Association

Disclosure: Received grant/research funds from Roche for investigator.

Acknowledgements

Ann Scheimann, MD, MBA Associate Professor, Department of Pediatrics, Section of Nutrition and Gastroenterology, Baylor College of Medicine and Johns Hopkins Medical Institution

Ann Scheimann, MD, MBA is a member of the following medical societies: North American Society for Pediatric Gastroenterology and Nutrition

Disclosure: Nothing to disclose.