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首页> 外文期刊>Current treatment options in gastroenterology >Surveillance and Treatment of Periampullary and Duodenal Adenomas in Familial Adenomatous Polyposis.
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Surveillance and Treatment of Periampullary and Duodenal Adenomas in Familial Adenomatous Polyposis.

机译：家族性腺瘤性息肉病的壶腹周围和十二指肠腺瘤的监测和治疗。

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Patients with familial adenomatous polyposis (FAP) have a cumulative lifetime risk of over 90% for developing duodenal adenomas, which are the precursor lesions for duodenal adenocarcinoma. Consequently, these patients have a 5% to 10% lifetime risk of periampullary or duodenal adenocarcinoma, making this the leading cause of cancer death in FAP patients who have had prophylactic colectomies. The increased relative risk of duodenal carcinoma in FAP patients and the poor outcomes associated with the treatment of advanced duodenal cancer have led to the development of prevention strategies for this cancer in the setting of FAP. It is generally accepted that surveillance for duodenal adenomas and adenocarcinomas should be included in the management of patients with FAP, although there are few data from clinical trials that demonstrate the effectiveness of surveillance strategies or chemoprevention for the prevention of death from duodenal cancer. Prospective case series have shown that endoscopic surveillance with endoscopic or surgical treatment of high-risk lesions in the duodenal or periampullary region can be performed with successful removal of the at-risk lesion(s). Surveillance should begin at about 21 years of age and should be performed using both an end-viewing and a side-viewing upper endoscope. An interval of 3 to 5 years between examinations appears to be adequate if no polyposis is evident. Once polyposis develops, an interval of 1 to 3 years between screenings for mild polyposis is appropriate. Patients with denser polyposis or larger adenomas are recommended to undergo examination every 6 to 12 months because of their increased risk of developing duodenal adenocarcinoma. Nonsteroidal anti-inflammatory drug therapy with sulindac, a nonselective cyclooxygenase (COX) inhibitor, or celecoxib, a COX-2 selective inhibitor, may be of benefit after the development of duodenal polyposis by inducing the regression or stabilization of the polyposis, although there is limited evidence from randomized, controlled trials to support its routine use. Almost all cases of adenocarcinoma occur in patients with advanced polyposis (Spigelman stage IV disease), and approximately 33% of this group will go on to develop adenocarcinoma if left untreated. The most definitive procedure for reducing the risk of adenocarcinoma is surgical resection of the ampulla and/or duodenum. Pancreaticoduodenectomy or pancreas-sparing duodenectomy are appropriate surgical therapies that are believed to substantially reduce the risk of developing periampullary adenocarcinoma. However, these procedures are associated with significant morbidity and mortality, including the risk of inducing desmoid tumor formation in FAP patients.
机译：家族性腺瘤性息肉病（FAP）患者发生十二指肠腺瘤的累积终生风险超过90％，十二指肠腺瘤是十二指肠腺癌的前体病变。因此，这些患者终生壶腹或十二指肠腺癌的风险为5％至10％，这使其成为患有预防性鞘膜切除术的FAP患者癌症死亡的主要原因。 FAP患者中十二指肠癌的相对风险增加以及与晚期十二指肠癌的治疗相关的不良结果已导致在FAP的背景下开发针对该癌症的预防策略。 FAP患者的治疗应包括十二指肠腺瘤和腺癌的监测，尽管临床试验中几乎没有数据表明监测策略或化学预防方法可预防十二指肠癌致死的有效性，这是公认的。前瞻性病例系列研究表明，在成功清除高危病变的同时，可以对十二指肠或壶腹周围区域的高危病变进行内窥镜检查或内镜或手术治疗。监视应从大约21岁开始，并且应同时使用端视和侧视上内窥镜进行。如果没有明显的息肉病，检查之间间隔3至5年就足够了。一旦发生息肉病，两次轻度息肉病之间的间隔时间为1至3年为宜。息肉病较重或腺瘤较大的患者建议每6到12个月进行检查，因为他们患十二指肠腺癌的风险增加。十二指肠息肉病发生后，使用舒林酸（一种非选择性环氧合酶（COX）抑制剂）或塞来昔布（一种COX-2选择性抑制剂）进行非甾体类抗炎药物治疗可能会受益，因为它可以诱导息肉消退或稳定，尽管存在来自随机对照试验的有限证据支持其常规使用。几乎所有腺癌病例都发生在晚期息肉病（Spigelman IV期疾病）患者中，如果不进行治疗，这一组中约有33％会继续发展为腺癌。降低腺癌风险的最确定的方法是手术切除壶腹和/或十二指肠。胰十二指肠切除术或保留胰十二指肠切除术是适当的外科治疗方法，据信可大大降低发生壶腹周围腺癌的风险。然而，这些程序与显着的发病率和死亡率相关，包括在FAP患者中诱发类胶体肿瘤形成的风险。

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