RESUMO
We report a rare carcinoma of the permanent ileostomy site developing 20 years or more after total proctocolectomy (TPC)in a 65-year-old woman with familial adenomatous polyposis(FAP). She underwent TPC for rectal cancer associated with FAP in her 40th at other institution. She also underwent pancreas-sparing total duodenectomy for duodenal mucosal cancer associated with severe duodenal polyposis at 59 years at our institution. She was referred to our hospital again complaining of the mass of the ileostomy site, 10 cm in diameter. Though biopsy revealed no definite malignancy, serum CA19-9 was elevated(98 U/mL), leading to a preoperative diagnosis to be ileal carcinoma. The involved bowel was widely resected. Histological examination demonstrated Stage â ¡A ileal carcinoma. Postoperative course was uneventful and she is well without recurrence 7 months after the ileal resection. This case seems valuable in that long-term surveillance including ileal carcinoma is important in the management of FAP patients whose colorectal cancer and duodenal cancer have been already well controlled.