Regular endoscopic surveillance and polypectomy is effective in managing rectal adenoma progression following colectomy and ileorectal anastomosis in patients with familial adenomatous polyposis

Abstract

Aim: Total colectomy with ileorectal anastomosis (TC-IRA) is a surgical option for patients with familial adenomatous polyposis (FAP). Regular endoscopic surveillance of the rectum is recommended to prevent rectal cancer. We aimed to document polyp progression in the rectum following TC-IRA and evaluate the role of polypectomy during surveillance. Method:Patients with FAP who underwent TC-IRA between 1990 and 2017 were identified. Demographic, endoscopic and genetic data were retrieved. Cumulative rectal adenoma (polyp) counts were obtained, whilst accounting for any polypectomies during the study period. The rate of polyp progression and factors influencing secondary proctectomy were evaluated.Results: One hundred and ninety-nine patients fulfilled our inclusion criteria, of which 44% were male. The median age at colectomy was 19 (range 11-70) years and median preoperative rectal polyp count was 7 (range 0-50). All patients had an APC pathogenic variant, of which 151 (79%) were 5' of the mutation cluster region (MCR), 19 (10%) in the MCR, six (3%) were 3' of the MCR and 15 (8%) had a gross deletion. After a median followup of 8.6 (range1-27) years and a median of 11 (range 2-37) flexible sigmoidoscopies per patient, the median rate of polyp progression was 5.5 polyps/year (range 0-70.2). There was no evidence of polyp regression. Eight (4%) patients underwent secondary proctectomy for neoplasia, of which one (0.5%) had rectal adenocarcinoma. A total of 13,527 polyps were removed, a median of 35 polyps/patient (range 0-829). The rate of polyp progression was not significantly associated with genotypic or phenotypic factors. Conclusion:Progression of rectal adenoma burden following TC-IRA appears to be slow and dependent on the length of follow-up. In the modern era of stringent endoscopic surveillance and therapeutic procedures such as cold snare polypectomy, the rate of secondary proctectomy and the risk of rectal cancer after TC-IRA are very low. These findings are important when counselling patients with regard to the choice of surgery for FAP and implementing endoscopic surveillance.

Prevalence and Management of Cancer of the Rectal Stump after Total Colectomy and Rectal Sparing in Patients with Familial Polyposis: Results from a Registry-Based Study

“…In our study population, the prevalence of cancer of the rectal stump was 6.57% with the median time frame between total colectomy with IRA and treatment of the rectal cancer being 13 years. This data suggests a slow adenoma to carcinoma progression, as recently stated in a study by St Mark’s Hospital based on their Polyposis Registry Population [ 23 ]. This one reports only one case of cancer of the rectal stump out of 191 patients after a median follow-up of 8.6 years.…”

“…In this study, more than half of the patients (64%) developed an early stage rectal cancer (A or B, according to Dukes’ classification). According to these data, recent minimally invasive techniques, such as TAMIS [ 35 ] and multistage polypectomy with flexible sigmoidoscopy [ 23 ], could be considered the main and resolute approach in most cases of cancer of the rectal stump. Our approach is in line with that of the Center for Hereditary Colorectal Neoplasia of Cleveland Clinic Foundation (Cleveland, Ohio) [ 36 ], with, in our opinion, narrower initial selective criteria, such as, for instance, the genetic background of the single patient, together with the phenotype and the clinical features.…”

Personalized endoscopic surveillance and intervention protocols for patients with familial adenomatous polyposis: the European FAP Consortium strategy

“…Obviously, patients with ISA/IRA stay at risk of developing adenomas and cancer in the retained rectum/rectosigmoid. Reported risks of rectal cancer after ISA/IRA vary between 0.5 % to 11.2 % during a median follow-up of 8.6 to 17.1 years 4 5 6 7 . Although overall 5.2 % to 11.7 % 4 7 undergo secondary proctectomy after ISA/IRA due to severe rectal polyposis or rectal cancer, one study in a historic series showed that at age 60 only in half of patients the rectum was still preserved 5 .…”

“…Reported risks of rectal cancer after ISA/IRA vary between 0.5 % to 11.2 % during a median follow-up of 8.6 to 17.1 years 4 5 6 7 . Although overall 5.2 % to 11.7 % 4 7 undergo secondary proctectomy after ISA/IRA due to severe rectal polyposis or rectal cancer, one study in a historic series showed that at age 60 only in half of patients the rectum was still preserved 5 . At 5, 10 and 15 years after IPAA, 7 % to 16 %, 35 % to 42 % and 75 % of patients will develop adenomas in the pouch, respectively 8 .…”

Is there a role for endoscopic management of the large bowel in familial adenomatous polyposis?

“…Most of this group had undergone colectomy and IRA, a familiar clinical scenario. Published data indicate that with endoscopic surveillance and polypectomy, long-term rectal preservation without cancer developing is possible [4,5]. There is a clear rationale for trying to avoid secondary proctectomy with the inherent complications related to pelvic dissection and the need for a stoma or ileoanal pouch, which adversely impact quality of life.…”