Incidence and Risk Factors of Cancer in the Anal Transitional Zone and Ileal Pouch following Surgery for Ulcerative Colitis and Familial Adenomatous Polyposis.

Guillaume Le Cosquer,Nathalie Vergnolle,Géraud Tuyeras,Louis Buscail,Cyrielle Gilletta,Céline Deraison,Barbara Bournet,Jean-Pierre Duffas,Etienne Buscail

doi:10.3390/cancers14030530

Abstract

Simple SummaryProctocolectomy with ileal pouch-anal anastomosis is the intervention of choice for ulcerative colitis and familial adenomatous polyposis requiring surgery. However high-grade dysplasia and cancer in the anal transitional zone and ileal pouch after 20 years is estimated to be 2 to 4.5% and 3 to 10% in ulcerative colitis and familial polyposis, respectively. The risk factors for ulcerative colitis are the presence of pre-operative dysplasia or cancer, disease duration > 10 years and severe villous atrophy. For familial polyposis, the risk factors are the number of pre-operative polyps > 1000, surgery with stapled anastomosis and the duration of follow-up. Even if anal transitional zone and ileal pouch cancers seldom occur following proctectomy for ulcerative colitis and familial adenomatous polyposis, the high mortality rate associated with this complication warrants close endoscopic monitoring, mainly every year with pouchoscopy including chromoendoscopy.Proctocolectomy with ileal pouch-anal anastomosis is the intervention of choice for ulcerative colitis and familial adenomatous polyposis requiring surgery. One of the long-term complications is pouch cancer, having a poor prognosis. The risk of high-grade dysplasia and cancer in the anal transitional zone and ileal pouch after 20 years is estimated to be 2 to 4.5% and 3 to 10% in ulcerative colitis and familial polyposis, respectively. The risk factors for ulcerative colitis are the presence of pre-operative dysplasia or cancer, disease duration > 10 years and severe villous atrophy. For familial polyposis, the risk factors are the number of pre-operative polyps > 1000, surgery with stapled anastomosis and the duration of follow-up. In the case of ulcerative colitis, a pouchoscopy should be performed annually if one of the following is present: dysplasia and cancer at surgery, primary sclerosing cholangitis, villous atrophy and active pouchitis (every 5 years without any of these factors). In the case of familial polyposis, endoscopy is recommended every year including chromoendoscopy. Even if anal transitional zone and ileal pouch cancers seldom occur following proctectomy for ulcerative colitis and familial adenomatous polyposis, the high mortality rate associated with this complication warrants endoscopic monitoring.

Highlights

Restorative proctocolectomy (RPC) with ileal pouch-anal anastomosis (IPAA) is the intervention of choice for familial adenomatous polyposis (FAP) and ulcerative colitis (UC) requiring surgery [1]
The phenotypic presentation is characterised by the early onset of hundreds of adenomas leading to colorectal cancer by the age of 40 in almost 100% of cases
Smith et al estimated that 75% of pouch-related cancers post-RPC with IPAA in FAP patients are located in the anal transitional zone (ATZ) [27]

Summary

Introduction

Restorative proctocolectomy (RPC) with ileal pouch-anal anastomosis (IPAA) is the intervention of choice for familial adenomatous polyposis (FAP) and ulcerative colitis (UC) requiring surgery [1]. The colectomy rate 10 years post-diagnosis has recently been estimated to be 15.6% [4]. FAP is an inherited autosomal dominant disease associated with predisposition to colorectal cancer, with a prevalence of 1 in 10,000 inhabitants [6]. This syndrome is caused by a germline mutation in the adenomatous polyposis coli gene (located on chromosome 5q21) [7]. Prophylactic colectomy is the only treatment to reduce the colorectal cancer risk and should be offered to all patients between 15 and 25 years of age [8]

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