Nine-year follow-up of a patient with attenuated familial adenomatous polyposis treated with cyclo-oxygenase-2 inhibitors

Familial adenomatous polyposis (FAP) is caused by germline mutations in the adenomatous polyposis coli (APC) gene with onset of florid polyposis in childhood and development of colorectal cancer by age 30. Colectomy is advised because of the high risk of developing colorectal cancer. Attenuated FAP (AFAP) is a variant of this condition with a later age of onset and milder clinical phenotype; however, colectomy is advised once polyposis develops and polyps cannot be managed endoscopically. We report a case of a patient with AFAP and previously resected colonic carcinoma that was treated with chemoprophylaxis with long-term cyclooxygenase-2 (COX-2) inhibitors after declining colectomy. Colonoscopic examination demonstrated regression of polyps by 18 months. After 9 years of follow-up, there was no evidence of colorectal cancer development or progression of polyposis. This is the first case report on long-term treatment with COX-2 inhibition in a patient with AFAP and previous colonic carcinoma.