Abstract
The incidence of familial adenomatous polyposis (FAP) is one in 7,000 to 12,000 live births. Virtually, all surgically untreated patients with FAP inevitably develop colorectal-cancer in their lifetime because they carry the adenomatous polyposis coli gene. Thus prophylactic proctocolectomy is indicated. Surgical treatment of FAP is still controversial. There are however, four surgical options: ileorectal anastomosis, restorative proctocolectomy with ileal pouch-anal anastomosis, proctocolectomy with ileostomy, and proctocolectomy with continent-ileostomy. Conventional proctocolectomy options largely lie between colectomy with ileorectal anastomosis or ileal pouch-anal anastomosis. Detractors of ileal pouch-anal anastomosis prefer ileorectal anastomosis because of better functional results and quality of life. The functional outcome of total colectomy with ileorectal anastomosis is undoubtedly far superior to that of the ileoanal pouch; however, the risk for rectal cancer is increased by 30%. Even after mucosectomy, inadvertent small mucosal residual islands remain. These residual islands carry the potential for the development of subsequent malignancy. We reviewed the literature (1975-2012) on the incidence, nature, and possible etiology of subsequent ileal-pouch and anal transit zone adenocarcinoma after prophylactic surgery procedure for FAP. To date there are 24 studies reporting 92 pouch-related cancers; 15 case reports, 4 prospective and 5 retrospective studies. Twenty three of 92 cancers (25%) developed in the pouch mucosa and 69 (75%) in anal transit zone (ATZ). Current recommendation for pouch surveillance and treatment are presented. Data suggest lifetime surveillance of these patients.
Highlights
Colorectal cancer remains a major problem in the treatment of patients with Familial adenomatous polyposis (FAP)
The functional outcome of total colectomy with ileorectal anastomosis is undoubtedly far superior to that of the ileoanal pouch; the risk for rectal cancer is increased by 30%
We reviewed the literature (1975-2012) on the incidence, nature, and possible etiology of subsequent ileal-pouch and anal transit zone adenocarcinoma after prophylactic surgery procedure for familial adenomatous polyposis (FAP)
Summary
Colorectal cancer remains a major problem in the treatment of patients with Familial adenomatous polyposis (FAP). Four surgical options are available for patients with FAP: [17,19,20] colectomy with ileorectal anastomosis (IRA), restorative proctocolectomy (RPC) with ileal pouch-anal anastomosis (IPAA), proctocolectomy with ileostomy, and proctocolectomy with continent ileostomy (Kock). While total colectomy with IRA provides superior functional results because it leaves the rectum intact, patients remain at a higher probability, compared to IPAA, of developing rectal cancer [2,27]. After IRA, 30% of FAP patients develop rectal cancer before the age of 60 years with an average mortality of about 25% [28]. This development relates to the time before the surgical interventions of the 1980s, when surgical options were much more limited. This review includes reports of carcinomas appearing in the residual rectal mucosa or anastomosis after IRA (10% - 31%) and in the ileal pouch body mucosa after Kock or IPAA (8% - 62%) [29,30,31,32,33,34,35,36,37,38]
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