Adenocarcinomas after Prophylactic Surgery for Familial Adenomatous Polyposis

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.

Postoperative complications after stapled and hand‐sewn ileal pouch‐anal anastomosis for familial adenomatous polyposis: A multicenter study

“…Hand‐sewn IPAA with mucosectomy is generally accepted as a time‐consuming and complicated procedure 25. Hence, in Western countries, this technique has been increasingly replaced by stapled IPAA without mucosectomy 3, 4, 5, 6.…”

Outcomes and complications of radiation therapy in patients with familial adenomatous polyposis

“…While colorectal cancer is the cause of death in up to 48% of patients dying with a known diagnosis of FAP, those who undergo prophylactic colectomy are more likely to die of desmoid tumors, and periampullary malignancies (12). Excision of the entire colorectal mucosa is the treatment of choice to avoid later rectal cancers (13); however, the timing of completion proctectomy has been a matter of debate (14). The risk of cancer in the retained rectum increases sharply from 10% for patients under 50 to 30% after the age of 60 years (15,16).…”

Surveillance of patients with hereditary gastrointestinal cancer syndromes

“…After surgery, annual endoscopic follow-up is recommended for patients with a rectal remnant, due to the risk of developing rectal cancer. In patients who have undergone proctocolectomy, reservoir endoscopic surveillance is essential due to the risk of developing adenomas (up to 60% of cases) [40,41].…”