Purpose: Total proctocolectomy (TPC) and end ileostomy has been considered the gold standard for surgical treatment of ulcerative colitis (UC) and familial adenomatous polyposis (FAP) since the early 1950s. Adenocarcinoma arising in an ileostomy is a late, rare complication. We report four cases of primary ileostomy adenocarcinoma (ICa) and review the literature.METHODSA medical records search was conducted at an academic medical center to identify patients with ileostomy adenocarcinoma. Clinical details (demographics, operative details, pathology, and outcomes) were obtained via chart review. Histopathology was reviewed. A Medline search of the English literature from 1969-2005 was then conducted for keywords: ileostomy, adenocarcinoma, UC, and FAP.
Results: Cases: 1) A 78 yo woman with UC s/p TPC in 1959 presented in 1998 with ICa; 2) A 76 yo woman with UC s/p TPC in 1982 presented in 1994 with ICa in a polyp. She re-presented in 2005 with a new Stage II ICa arising from a tubulovillous adenoma; 3) A 62 yo woman with FAP s/p TPC in 1971 presented in 2005 with Stage I ICa in a villous adenoma; 4) A 58 yo male with UC s/p TPC in 1971 presented in 2006 with ICa arising from a polyp. Each patient underwent wide ileostomy excision with abdominal wall margin and adjacent/ileum mesentery followed by stomal resiting. Each is without evidence of recurrent disease at most recent follow-up (79 months, 10 months, 9 months, and 1 month, respectively). Average time from stoma creation to ICa was 27 ± 15 years. Review of the literature identified 50 cases of ICa with an average interval of 26 years from stoma creation to ICa.
Conclusions: Our four cases were similar to those found in the literature in terms of average interval to ICa formation, ratio of occurrence in UC and FAP, and formation as a polypoid mass in the majority. One of our patients represents the first reported case of a metachronous primary ICa. Ileostomy excision with abdominal wall margin and stomal resiting is the treatment of choice with low recurrence rates for early ICa and low morbidity/mortality. Ileostomy polyps should be excised, and ileostomies should be surveilled for ICa yearly starting at 10 years after TPC/ileostomy formation.