Ileal pouch adenomas and carcinomas after restorative proctocolectomy for familial adenomatous polyposis.
ABSTRACT Restorative proctocolectomy and IPAA has become the treatment of choice in familial adenomatous polyposis. However, several cases of adenomas and carcinomas arising in the ileal pouch were reported.
The aim of this study was to evaluate the prevalence and natural history of ileal pouch adenomas and the development of carcinomas in patients with restorative proctocolectomy for familial adenomatous polyposis.
We prospectively studied patients who underwent IPAA during the past 20 years at the surgical unit of the University of Florence in Italy.
We investigated the extent of the risk and the factors that are involved in the development of neoplastic changes of the pouch. Furthermore, because it is not entirely clear when and how polyps should be treated, we have revised our modality of treatment for this unusual pathology.
Sixty-nine patients with familial adenomatous polyposis underwent restorative proctocolectomy. In 66 patients, handsewn ileoanal anastomosis with anal canal mucosectomy was performed. After surgery, all patients underwent endoscopic surveillance.
After 10 years of follow-up, 1 ileal pouch adenoma was found in 64.9% of restorative proctocolectomy patients, and ileal pouch carcinomas occurred in 2 patients (29 and 59 years old), 3 and 11 years after restorative proctocolectomy. The number of colonic adenomatous polyps influenced the occurrence of pouch adenomas. No patients with <200 colonic adenomas experienced pouch adenomas, but 46% of patients with >1000 colonic polyps had pouch adenomas, and 25% of patients with 200 to 1000 colonic polyps had pouch adenomas at follow-up. No relationship was found between ileal pouch adenomas and pouch shape (J, S, or straight ileoanal anastomosis with multiple myotomies) or the APC mutation. Polyps larger than 5 mm were removed by endoscopy or surgery.
Ileal pouch adenomas were common after restorative proctocolectomy. Patients >50 years of age and patients with >1000 colonic adenomas at the time of colectomy were more prone to ileal pouch adenomas. The development of malignancy in the terminal ileum can present a fast course and does not seem to follow the classic adenoma-carcinoma sequence.
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ABSTRACT: Familial adenomatous polyposis (FAP) is an autosomal dominant inherited syndrome characterized by multiple adenomatous polyps (predisposing to colorectal cancer development) and numerous extracolonic manifestations. The underlying genetic burden generates variable clinical features that may influence operative management. As a precancerous hereditary condition, the rationale of performing a prophylactic surgery is a mainstay of FAP management. The purpose of the present paper is to bring up many controversial aspects regarding surgical treatment for FAP, and to discuss the results and perspectives of the operative choices and approaches. Preferably, the decision-making process should not be limited to the conventional confrontation of pros and cons of ileorectal anastomosis or restorative proctocolectomy. A wide discussion with the patient may evaluate issues such as age, genotype, family history, sphincter function, the presence or risk of desmoid disease, potential complications of each procedure and chances of postoperative surveillance. Therefore, the definition of the best moment and the choice of appropriate procedure constitute an individual decision that must take into consideration patient's preferences and full information about the complex nature of the disease. All these facts reinforce the idea that FAP patients should be managed by experienced surgeons working in specialized centers to achieve the best immediate and long-term results.World Journal of Gastroenterology 11/2014; 20(44):16620-16629. · 2.43 Impact Factor
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ABSTRACT: Restorative proctocolectomy is the most common surgical option for patients with familial adenomatous polyposis (FAP). However, adenomas may develop in the ileal pouch mucosa over time, and even carcinoma in the pouch has been reported. We therefore reviewed the prevalence, nature, and treatment of adenomas and carcinoma that develop after proctocolectomy in the ileal pouch mucosa in patients with FAP. In 25 reports that were reviewed, the incidence of adenomas in the ileal pouch varied from 6.7% to 73.9%. Several potential factors that favor the development of pouch polyposis have been investigated, but many remain controversial. Nevertheless, it seems certain that the age of the pouch is important. The risk appears to be 7% to 16% after 5 years, 35% to 42% after 10 years, and 75% after 15 years. On the other hand, only 21 cases of ileal pouch carcinoma have been recorded in the literature to date. The diagnosis of pouch carcinoma was made between 3 to 20 years (median, 10 years) after pouch construction. Although the risk of malignant transformation in ileal pouches is probably low, it is not negligible, and the long-term risk cannot presently be well quantified. Regular endoscopic surveillance, especially using chromoendoscopy, is recommended.World Journal of Gastroenterology 10/2013; 19(40):6774-6783. · 2.43 Impact Factor
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ABSTRACT: Familial adenomatous polyposis (FAP) is an autosomally dominant disease characterized by the early development of colorectal adenomas and carcinoma in untreated patients. Patients with FAP may develop rectal cancer at their initial presentation (primary) or after prophylactic surgery (secondary). Controversies exist regarding which surgical procedure represents the best first-line treatment. The options for FAP are ileorectal anastomosis (IRA) or a restorative proctocolectomy (RPC) with either a handsewn or a stapled ileal pouch-anal anastomosis (IPAA), with or without mucosectomy. The purpose of these surgeries is to stop progression to an adenoma-cancer sequence by eradicating the colon, a disease prone organ. Unfortunately, these surgical procedures, which excise the entire colon and rectum while maintaining transanal fecal continence, do not guarantee that patients still won't develop adenomas. Based on the available literature, we therefore reviewed reported incidences of pouch-related adenomas that occurred post prophylactic surgery for FAP. The review consists of a collection of case, descriptive, prospective and retrospective reports. To provide available data on the natural history of subsequent adenomas after prophylactic surgery (by type) for FAP. A review was conducted of existing case, descriptive, prospective and retrospective reports for patients undergoing prophylactic surgery for FAP (1975 - August, 2013). In each case, the adenomas were clearly diagnosed in one of the following: the ileal pouch mucosa (above the ileorectal anastomosis), within the anorectal segment (ARS) below the ileorectal anastomosis, or in the afferent ileal loop. A total of 515 (36%) patients with pouch-related adenomas have been reported. Two hundred and eleven (211) patients had adenomas in the ileal pouch mucosa, 295 had them in the ARS and in 9 were in the afferent ileal loop. Patients with pouch adenomas without dysplasia or cancer were either endoscopically polypectomized or were treated with a coagulation modality using either a Nd:Yag laser or argon plasma coagulation (as indicated). Patients with dysplastic pouch adenomas or pouch adenomas with cancer had their pouch excised (pouchectomy). In patients with FAP treated with IRA or RPC with IPAA, the formation of adenomas in the pouch-body mucosa or ARS/anastomosis and in the afferent ileal loop is apparent. Because of risks for adenoma recurrence, a life time endoscopic pouch-surveillance is warranted.The world journal of colorectal surgery. 01/2013; 3(2).