Rectal Cancer Following Colectomy and Ileorectal Anastomosis for Familial Adenomatous Polyposis

Abstract

Background: Familial adenomatous polyposis (FAP) has historically been treated by colectomy and ileorectal anastomosis (IRA). Preservation of the rectum allows the subsequent development of cancer in the rectum. The risk of rectal cancer following ileorectal anastomosis in the Australian population has not been published to date. Methods: An audit of the Familial Adenomatous Polyposis Registry of Western Australia was undertaken to assess patients who had undergone colectomy and ileorectal anastomosis. Fifty-five patients ranging in age from 13 to 65 years were studied. Results: Seven patients (13%) developed cancer of the rectum with a median follow-up of 10 years (range: 1-31 years). Median interval to diagnosis of carcinoma of the rectum following colectomy and IRA was 10 years. All patients who developed cancer in the retained rectum had rectal polyps. Colon cancer was present in the initial colectomy specimen in 13 patients (of these, five patients developed rectal cancer). Flat polyps were noted in five patients. Four patients with flat polyps developed cancer of the rectum. Conclusions: Total colectomy and IRA should be considered as part 1 of a staged procedure in the patient with FAP. With the exception of the patient with no evidence of rectal polyps, completion proctectomy should be undertaken within 10 years of the initial colectomy.

Aneuploidy Arises at Early Stages of Apc-Driven Intestinal Tumorigenesis and Pinpoints Conserved Chromosomal Loci of Allelic Imbalance between Mouse and Human

“…The localization of the Stk11 gene within this region is of particular interest in view of the causative role of this serine/threonine kinase in Peutz-Jeghers syndrome, an inherited susceptibility to intestinal cancer characterized by multiple hamartomatous polyps throughout the upper GI tract and a high risk of developing cancer in the intestine and other organs. 53 To further restrict the size of the chromosomal intervals affected by gain/loss events and pinpoint specific conserved genes that play rate-limiting roles in intestinal adenoma formation in the mouse and human, we compared array CGH profiles from early FAP adenomas (obtained from carriers of known APC germline mutations) and from histology-matched intestinal tumors from Apc ϩ/1638N mice. The results of this comparative analysis (Table 2) have indicated the presence of a surprisingly high number of chromosomal aberrations shared between mouse upper GI and human colorectal adenomas, notwithstanding the different anatomical location of these two groups of GI tumors.…”

Efficacy and safety of eflornithine (CPP-1X)/sulindac combination therapy versus each as monotherapy in patients with familial adenomatous polyposis (FAP): design and rationale of a randomized, double-blind, Phase III trial

“…Individuals with extensive rectal involvement usually undergo total proctocolectomy with ileal-pouch anal anastomosis (IPAA) [ 3 ]. Many such patients develop progressive polyposis in the retained rectum [ 6 , 7 ] or ileal pouch [ 8 – 10 ]. Despite excision of the main at-risk organ(s), many patients develop duodenal adenomas and require frequent surveillance and endoscopic or surgical intervention [ 11 ].…”

Endoscopic management of patients with familial adenomatous polyposis after prophylactic colectomy or restorative proctocolectomy – systematic review of the literature

“…Jenner et al . 21 only included patients with a confirmed mutation. Five studies reported a cumulative incidence of rectal cancer ranging from 3% 22 to 17.2% 19 at 5 years, 7.7% 23 to 24.1% 19 at 10 years, 11% 22 to 23% 23 at 20 years, and 24% 22 at 30 years after the primary IRA.…”