Familial adenomatous polyposis(FAP) has a significant risk of developing colorectal cancer. As a consequence, their surgical treatment is currently widely performed by restorative proctocolectomy with ileal pouch-anal anastomosis(IPAA). Frequently, the potential for recurrence in FAP patients after above operation was overlooked. In fact, several reports were presented for warning of recurred cancer in ileal pouch. We report a case of a patient who developed adenocarcinoma in an ileal pouch after restorative proctocolectomy for familial adenomatous polyposis with a review of literature.
Familial adenomatous polyposis(FAP) is an inherited autosomal dominant disorder characterized by the appearance in early life of numerous adenomas of colon and rectum.
According to the recent studies, familial polypolis is a disease which produces polypoid lesions not only in the large intestine but also in the stomach duodenum, ileum and jejunum. The natural course of extracolonic lesions is variable, but the colonic polyps are associated with high incidence of cancer. The risk of malignant change is virtually 100% if untreated. The authors report a case of FAP in a 36-year-old-female who presented colorectal polyposis with multiple adenomas of the gallbladder. A review of literature with emphasis on the association of adenoma of the gallbladder was made.
Familial adenomatous polyposis(FAP) is a hereditary disorder characterized by the development in adolescence or early adult life of multiple adenomatous polyps throughout the colon and rectum. The risk of malignant transformation is so high as to virtually 100 per cent if the patient lives long enough. Gardner's syndrome, a variant of familial adenomatous polyposis, is characterized by colorectal adenomas, multiple osteomas and variety of soft tissue lesions, including sebaceous cysts, fibromas, lipomas, and desmoid tumors. The recent study show that this group of diseases are caused by different mutations of the same gene located on the long arm of chromosome 5. All family members at risk of inheriting this gene need frequent surveillance of the colon. This should start between the ages of 8 and 12 years and should be continued at regular intervals. Prompt colectomy is indicated at the time the diagnosis is made. We experienced a case of Gardner's syndrome, which had typical extracolonic manifestations. Herein, we report this case with a review of the literature.
Familial polyposis is a rare genetic disorder which the large intestine is diffusely carpeted by numerous adenomatous polyps. According to the recent studies, familial polyposis is a disease which produces polypoid lesions not only in the large intestine but also in the stomach, duodenum, ileum and jejunum. The natural course of extracolonic lesions is variable, but the colonic polyps are associated with high incidence of cancer. The risk of malignant change is virtually 100% if untreated. Thus when adenomatosis is noted, familial members at risk for familial adenomatous polyposis must be screened and prophylactic surgery performed to prevent inevitable colon cancer.
Controversy exists about the most appropriate prophylactic treatment. Because of many disadvantages of ileostomy, total colectomy with ileorectal anastomosis (ileoproctostomy) or total colectomy with rectal mucosa stripping and ileoanal anastomosis has become a more preferred approach. After ileorectal anastomosis, polyps in the retained rectum must be removed by endoscopic polypectomy and rigorously followed by sigmoidoscopy every few months for prevention of polyp reccurence or malignant transformation. We experienced three cases of familial adenomatous polyposis and 2 of them were treated successfully by preoperative colonoscopic polypectomy, total colectomy and ileoproctostomy.