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• W2891282410 abstract "Familial adenomatous polyposis (FAP) is an autosomal dominantly inherited syndrome caused by a germline mutation in the APC gene that accounts for approximately 1% of colorectal cancer cases in the United States. Mutations between codons 169-1393 result in classic FAP, whereas mutations closer to the 5’ and 3’ of the APC gene cause attenuated FAP, which has a less severe phenotype.1Vogelstein B. Kinzler K.W. The genetic basis of human cancer. McGraw-Hill, Health Professions Division, New York1998Google Scholar FAP is characterized by the development of multiple adenomatous colorectal polyps in childhood and adolescence, which inevitably progresses to colorectal carcinoma. The treatment of patients with FAP has been focused on preventing colorectal cancer by establishing colonoscopy-based surveillance strategies and early referral for prophylactic proctocolectomy. The surgical options available include colectomy with ileorectal anastomosis, and total proctocolectomy with mucosal proctectomy and ileoanal pull-through.2Giardiello F.M. Petersen G.M. Brensinger J.D. et al.Hepatoblastoma and APC gene mutation in familial adenomatous polyposis.Gut. 1996; 39: 867-869Crossref PubMed Scopus (130) Google Scholar, 3Romans K HL, Booker SV, Brensinger JD, et al. Hereditary Colorectal Cancer Website: http://www.hopkins-coloncancer.org.2000/.Google Scholar, 4Phillips RKS SA Thomson J.P.S. Familial adenomatous polyposis and other polyposis syndromes. Edward Arnold, London1994Google Scholar Owing to the risk of cancer in the retained rectal segment, surgical approaches that eliminate the rectum are advocated.4Phillips RKS SA Thomson J.P.S. Familial adenomatous polyposis and other polyposis syndromes. Edward Arnold, London1994Google Scholar Because prophylactic colectomy or proctocolectomy carries short-term and long-term adverse events, there has always been a desire to delay or prevent surgery through the use of endoscopic surveillance and chemopreventive agents. The ultimate desire for patients and providers would be the ability to convert FAP from a surgical to a medically managed condition. The study by Sarvepalli et al5Sarvepalli S. Burke C.A. Monachese M. et al.Natural history of colonic polyposis in young patients with familial adenomatous polyposis.Gastrointest Endosc. 2018; 88: 726-733Abstract Full Text Full Text PDF PubMed Scopus (17) Google Scholar examines the natural history of colonic polyposis in a cohort of 168 young patients (<30 years of age) with FAP and sheds light on their rate of polyposis progression. The rate of polyposis progression among patients who did not receive chemopreventive agents was 25 polyps per year, with the highest median rate of polyposis progression associated with mutations located between codons 1309 and 1389 (mutation cluster region). The lowest rate of polyposis progression was noted among patients with mutations between codons 93 and 151 (attenuated FAP). Furthermore, progression of polyposis was also associated with baseline polyp burden, with patients with <20 colonic polyps having a significantly lower rate of polyposis progression than did those with ≥20 polyps. In their FAP cohort, 40% of patients underwent surgery after a median of 3.5 years of endoscopic surveillance. The results from their work provide the first data on polyposis progression among young FAP patients, and they identify independent risk factors of disease progression, which may be used by practitioners to provide an initial assessment and aid in assesing the risk of progression of disease and management. The treatment of patients with FAP has incorporated the use of chemopreventive agents to decrease colorectal polyposis burden, thereby reducing the morbidity associated with the disease. The use of nonselective and selective cyclooxygenase (COX-2) inhibitors (sulindac and celecoxib, respectively) to prevent or induce the regression of polyps in the retained rectum of FAP patients has been shown to be effective in short-term trials and in long-term sulindac studies.6Nugent K.P. Phillips R.K. Rectal cancer risk in older patients with familial adenomatous polyposis and an ileorectal anastomosis: a cause for concern.Br J Surg. 1992; 79: 1204-1206Crossref PubMed Scopus (131) Google Scholar, 7Giardiello F.M. Hamilton S.R. Krush A.J. et al.Treatment of colonic and rectal adenomas with sulindac in familial adenomatous polyposis.N Engl J Med. 1993; 328: 1313-1316Crossref PubMed Scopus (1552) Google Scholar, 8Cruz-Correa M. Hylind L.M. Romans K.E. et al.Long-term treatment with sulindac in familial adenomatous polyposis: a prospective cohort study.Gastroenterology. 2002; 122: 641-645Abstract Full Text Full Text PDF PubMed Scopus (229) Google Scholar, 9Steinbach G. Lynch P.M. Phillips R.K. et al.The effect of celecoxib, a cyclooxygenase-2 inhibitor, in familial adenomatous polyposis.N Engl J Med. 2000; 342 (1946-2)Crossref PubMed Scopus (2280) Google Scholar However, FAP patients exposed to chemoprevention may still experience colorectal cancer.8Cruz-Correa M. Hylind L.M. Romans K.E. et al.Long-term treatment with sulindac in familial adenomatous polyposis: a prospective cohort study.Gastroenterology. 2002; 122: 641-645Abstract Full Text Full Text PDF PubMed Scopus (229) Google Scholar, 10Lynch H.T. Thorson A.G. Smyrk T. Rectal cancer after prolonged sulindac chemoprevention: a case report.Cancer. 1995; 75: 936-938Crossref PubMed Scopus (98) Google Scholar, 11Giardiello F.M. Spannhake E.W. DuBois R.N. et al.Prostaglandin levels in human colorectal mucosa: effects of sulindac in patients with familial adenomatous polyposis.Dig Dis Sci. 1998; 43: 311-316Crossref PubMed Scopus (95) Google Scholar, 12Giardiello F.M. Yang V.W. Hylind L.M. et al.Primary chemoprevention of familial adenomatous polyposis with sulindac.N Engl J Med. 2002; 346: 1054-1059Crossref PubMed Scopus (348) Google Scholar The administration of sulindac for primary chemoprevention of FAP failed to prevent the development of adenomatous polyposis in unaffected gene mutation carriers.13Phillips R.K. Wallace M.H. Lynch P.M. et al.A randomised, double blind, placebo controlled study of celecoxib, a selective cyclooxygenase 2 inhibitor, on duodenal polyposis in familial adenomatous polyposis.Gut. 2002; 50: 857-860Crossref PubMed Scopus (375) Google Scholar Consequently, treatment with nonsteroidal anti-inflammatory drugs (NSAIDs) is not routinely recommended for presymptomatic gene carriers, nor are these drugs advocated for the primary treatment of polyposis in patients with intact colons. In the current study,5Sarvepalli S. Burke C.A. Monachese M. et al.Natural history of colonic polyposis in young patients with familial adenomatous polyposis.Gastrointest Endosc. 2018; 88: 726-733Abstract Full Text Full Text PDF PubMed Scopus (17) Google Scholar the rate of polyposis progression was significantly lower among patients exposed to NSAID-based chemoprevention compared with those not exposed (P < .0005), and exposure to chemoprevention was an independent factor for reduction of polyposis progression regardless of APC mutation. These data support the role of chemoprevention in young patients with FAP and and of its use as an alternative for patients with a high risk of polyposis progression as a strategy to delay referral for surgery. Of note, progression to colorectal cancer occurred in 1 in 690 patient-years of surveillance, in a patient with a very high rate of polyposis (101.6 polyps/year).5Sarvepalli S. Burke C.A. Monachese M. et al.Natural history of colonic polyposis in young patients with familial adenomatous polyposis.Gastrointest Endosc. 2018; 88: 726-733Abstract Full Text Full Text PDF PubMed Scopus (17) Google Scholar Other factors that have traditionally been considered risk factors for progression of disease, such as location of polyps, presence of advanced histologic features, type of mutation, and family history of colorectal cancer, were not associated with progression of disease in their study. In our practice, we have reserved chemopreventive agents for children with a more agresive FAP phenotype based on polyp number at baseline so as to delay surgical management and for control of rectal polyps after ileorectal anastomosis in the adult population. The data from this study add to the current body of evidence supporting the use of chemoprevention as part of the treatment of young FAP patients to delay referral to surgery for asymptomatic patients. Clinicians may consider the use of chemoprevention early in the treatment of young FAP patients who present with a high polyp burden (>20), those with mutations located within a specific cluster region, or both. Other non-NSAID chemopreventive agents with less toxicity have been proposed, including oral bioflavinoids. Our group recently completed a phase IIB randomized clinical trial examining the role of oral curcumin as a chemopreventive agent in patients with FAP and did not find a statistically significant reduction in colorectal polyp burden associated with the use of oral curcumin.14Cruz-Correa M, Hylind LM, Marrero JH, et al. Efficacy and safety of curcumin in treatment of intestinal adenomas in patients with familial adenomatous polyposis. Gastroenterology. Epub 2018 May 23.Google Scholar A recent study by Lynch et al15Lynch P.M. Burke C.A. Phillips R. et al.An international randomised trial of celecoxib versus celecoxib plus difluoromethylornithine in patients with familial adenomatous polyposis.Gut. 2016; 65: 286-295Crossref PubMed Scopus (63) Google Scholar examined the efficacy of oral difluoromethylornithine (DFMO), an irreversible enzyme-activated inhibitor of ornithine decarboxylase, as chemoprevention for colorectal polyps in patients with FAP. DFMO has been associated with suppression of tumor development in animal models and inhibition of cell proliferation. The use of DFMO in combination with celecoxib (selective COX-2 inhibitor) was found to significantly reduce colorectal polyp burden with the use of a video-based global polyp index measurement (P = .03) compared with the use of celecoxib alone. Furthermore, the investigators reported no cases of toxicity among patients receiving DFMO and good tolerability of the study agents. Chemoprevention continues to be an area of intense research; multiple agents are currently being studied in clinical trials aimed at decreasing polyp burden in FAP patients (Clinical Trials.gov). A risk-stratified approach for disease management with early surgical referral for young FAP patients with a high risk of polyposis progression and colonoscopy surveillance with or without chemoprevention for those with a low risk of progression may provide a sensible option and warrants additional research. Randomized clinical trials including FAP patients from all ages, incorporating data on environmental exposures, and using validated measurements including video-based polyp measurements15Lynch P.M. Burke C.A. Phillips R. et al.An international randomised trial of celecoxib versus celecoxib plus difluoromethylornithine in patients with familial adenomatous polyposis.Gut. 2016; 65: 286-295Crossref PubMed Scopus (63) Google Scholar to capture clinical response, are key to assess polyposis progression or regression, and to advance the field by providing the best alternatives for patients with FAP. Clinical guidelines for management of FAP recommend that surgery be performed if colorectal cancer or symptoms are present,16Syngal S. Brand R.E. Church J.M. et al.ACG clinical guideline: genetic testing and management of hereditary gastrointestinal cancer syndromes.Am J Gastroenterol. 2015; 110 (quiz 263): 223-262Crossref PubMed Scopus (914) Google Scholar but the indications in asymptomatic patients are less clear. Referral to surgical management is recommended for FAP patients who have “a significant increase in polyp burden,” but no specific polyp burden has been established, and clinical practice decisions reflects local expertise and the patient’s and physician’s preferences. The current study provides data to support a risk-stratified approach to evaluating FAP in young patients and to guide their treatment, including surveillance colonoscopy, chemoprevention, or both for FAP patients who have a lower risk for polyposis progression. All authors disclosed no financial relationships relevant to this publication. Natural history of colonic polyposis in young patients with familial adenomatous polyposisGastrointestinal EndoscopyVol. 88Issue 4PreviewProctocolectomy prevents colorectal cancer in familial adenomatous polyposis (FAP). Colorectal polyp progression is one of the indications for surgery. No data exist regarding the natural history of colorectal polyposis in young patients with FAP. This study examined the rate of polyposis progression and factors associated with it. Full-Text PDF" @default.
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• W2891282410 title "Reassessing colectomy in young patients with familial adenomatous polyposis" @default.
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