Original article—alimentary tractRisk of Developing Adenomas and Carcinomas in the Ileal Pouch in Patients With Familial Adenomatous Polyposis
Section snippets
Dutch Polyposis Registry
In 1985, the Netherlands Foundation for the Detection of Hereditary Tumours (NFDHT) established a registry of patients with FAP. The main objective of the registry is to promote the early detection of cancer in high-risk families. The approach and the regulations of the registry have been described elsewhere.19 In short, families with FAP are referred to the national registry by clinical geneticists, surgeons, or gastroenterologists. During the early years, social workers and genetic field
Results
Between 1984 and 2005, 327 families with FAP were registered at the NFDHT. A total of 254 FAP patients (141 male, 113 female) with an IPAA were identified within these families. In 40 patients, 15.7% of the total cohort, no endoscopy surveillance was performed, or the endoscopy or pathology reports could not be obtained. In 2 patients, a pouch carcinoma was diagnosed 5 years after reconstructive proctocolectomy for a primary rectal cancer. Because we could not exclude that the pouch carcinoma
Discussion
The present study provides for the first time an estimate of the cumulative risk of developing a pouch carcinoma in patients with FAP. In this cohort of 212 patients with FAP prospectively followed by the Dutch FAP polyposis registry, the cumulative risk for adenoma development in the pouch after 5- and 10-year follow-up was 16.0% and 42.2%, respectively. In contrast, the risk of developing a carcinoma in the pouch was only 1% at 10-year follow-up. We also demonstrated that the use of
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Management of pouch neoplasia: consensus guidelines from the International Ileal Pouch Consortium
2022, The Lancet Gastroenterology and HepatologyCitation Excerpt :In the 212 patients with FAP and IPAA in the Dutch Polyposis Registry, the cumulative risk of developing an adenoma in the pouch at 10-year follow-up was 45%. However, 25 (75·7%) of 33 adenomas were found in a subgroup of patients who were examined with chromoendoscopy, compared with 74 (34·9%) of 212 adenomas of the whole cohort surveyed using white-light endoscopy with or without chromoendoscopy.34 For dysplasia surveillance in FAP, pouchoscopy is often performed along with upper gastrointestinal endoscopy to screen for gastroduodenal polyps, neoplasias, or malignancies.
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2022, Best Practice and Research: Clinical GastroenterologyCitation Excerpt :The incidence of rectal cancer drastically decreased since the introduction of IPAA in 1978, probably since before patients with severe rectal polyposis underwent IRA and were at higher risk of developing rectal cancer [40]. The incidence of developing cancer after IPAA in FAP is 1.1–1.9% [38,41]. Most of these cancers are located in the rectal cuff rather than in the pouch body itself.
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2021, The Lancet Gastroenterology and HepatologyAmerican Society for Gastrointestinal Endoscopy guideline on the role of endoscopy in familial adenomatous polyposis syndromes
2020, Gastrointestinal EndoscopyCitation Excerpt :This study found improved long-term survival in patients who pursued IPAA with no difference in short-term outcomes including postoperative adverse events when compared with patients undergoing colectomy with IRA, which is likely related to the long-term risk of rectal cancer.63 There is an increased risk of adenomas in the ileum, rectal cuff, and anal transition zone after colectomy and IPAA and IRA; therefore, surveillance after surgery is necessary.64-72 Friedrich et al69 showed a 45% cumulative risk of developing an adenoma in the pouch 10 years after proctocolectomy with IPAA.
The authors disclose no financial conflicts of interest.