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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Cited by (60)
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.
Management of familial adenomatous polyposis and MUTYH-associated polyposis; new insights
2022, Best Practice and Research: Clinical GastroenterologyCitation Excerpt :Currently the use of a personalized endoscopic surveillance and intervention protocol for patients with FAP and IRA/ISA or IPAA is studied in a multi-center prospective study (NCT04678011). Albeit studies on the use of advanced imaging techniques after colectomy are scarce, the use of virtual or dye chromoendoscopy next to white light endoscopy seems to result in a more accurate assessment than the use of white light endoscopy alone [41]. The life-time risk of developing duodenal adenomas approaches 100% in FAP compared to lower rates reported at 21% in MAP [62–64].
Ileoanal pouch cancers in ulcerative colitis and familial adenomatous polyposis: A systematic review and meta-analysis
2022, Digestive and Liver DiseaseEndoscopic Management and Surgical Considerations for Familial Adenomatous Polyposis
2022, Gastrointestinal Endoscopy Clinics of North AmericaCitation Excerpt :In patients undergoing IPAA, subsequent cancer risk in the pouch is about 1%, with most (75%) cancers developing in the anal transition zone.24 The cumulative risk for adenoma development in the pouch may be as high as 45% (including 12% with advanced histology), and ongoing endoscopic surveillance with polypectomy is recommended.25 Guidelines recommend endoscopic surveillance in accordance with the type of colectomy (Table 2).2–5
Diagnosis and classification of ileal pouch disorders: consensus guidelines from the International Ileal Pouch Consortium
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.