Original article—alimentary tract
Risk of Developing Adenomas and Carcinomas in the Ileal Pouch in Patients With Familial Adenomatous Polyposis

https://doi.org/10.1016/j.cgh.2008.06.011Get rights and content

Background & Aims

At present, more than half of patients with familial adenomatous polyposis (FAP) are treated with a proctocolectomy and an ileal pouch–anal anastomosis (IPAA). Originally it was thought that this procedure would eliminate the risk of developing rectal cancer. However, an increasing number of studies reported development of adenoma and carcinoma in the pouch. The aim of this study was to evaluate the long-term risk of developing adenomas and carcinomas in the pouch in a large cohort of Dutch FAP patients.

Methods

A total of 254 patients with FAP who underwent an IPAA were selected from the Dutch Polyposis Registry. The results of the surveillance examinations and the pathology reports were analyzed. Surveillance with chromoendoscopy was offered to a subgroup of patients.

Results

Full information on follow-up was available in 212 (84%) patients. These patients (56% male) underwent a total of 761 endoscopies. The mean follow-up was 7.9 years (range, 0.4–20.3 years). The cumulative risk of developing an adenoma in the pouch at 10-year follow-up was 45%. Twenty-five patients (11.8%) developed an adenoma with advanced pathology, and 4 (1.9%) developed a carcinoma. The cumulative risk of developing a pouch carcinoma at 10-year follow-up was 1%. A very high prevalence (75.7%) of adenomas was found in a subgroup of patients who were examined with chromoendoscopy.

Conclusions

This study demonstrated that although the risk of developing adenomas in the pouch after an IPAA is high, the risk of malignant degeneration appears to be low. The use of chromoendoscopy improves the detection of small adenomas.

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

References (21)

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The authors disclose no financial conflicts of interest.

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