Frequency and Features of Duodenal Adenomas in Patients With MUTYH-Associated Polyposis

Frequency and Features of Duodenal Adenomas in Patients With MUTYH-Associated Polyposis
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DOI:
10.1016/j.cgh.2016.02.020
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发表时间:
2016-07-01
影响因子:
12.6
通讯作者:
Latchford, Andrew
Latchford, Andrew
中科院分区:
医学1区
文献类型:
--
作者:
Walton, Sarah-Jane;Kallenberg, Frank G. J.;Latchford, Andrew

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背景与目的:mutyh相关性息肉病(MAP)与家族性腺瘤性息肉病(FAP)相似,都增加了发生十二指肠腺瘤和癌症的风险。几乎所有FAP患者发展为十二指肠腺瘤,5%发展为十二指肠癌。关于MAP患者中十二指肠腺瘤和癌症的患病率知之甚少,但目前对fap患者的监测建议是相同的-他们应该在25岁时开始监测。我们的目的是评估MAP患者十二指肠腺瘤的患病率、程度和进展,并评估上胃肠道监测的建议。方法:在一项回顾性研究中,我们收集了2002年至2014年在圣马克医院(英国伦敦)和学术医学中心(荷兰阿姆斯特丹)登记的所有MAP患者(n = 92)进行食管胃十二指肠镜检查的数据。我们收集了有关腺瘤发展、腺瘤发现年龄、干预措施和疾病进展的信息。结果:31例(34%)患者检测到十二指肠腺瘤,中位年龄50岁。当首次发现十二指肠息肉病时,84%的患者为Spigelman I期或II期;大多数有少量小息肉,无高度发育不良或绒毛特征。随后对其中18例患者进行食管胃十二指肠镜检查,发现14例(78%)为Spigelman 0 - II期疾病(中位随访时间为7.8年)。6例患者因病变大小或绒毛特征而分期进展,病程超过9.5年(2例达到IV期)。在活检或息肉切除术分析后,8例患者的腺瘤分期下降,3例患者的腺瘤分期不变。结论:在一项来自92例MAP患者的数据分析中,十二指肠息肉病的发生频率似乎低于FAP患者,且发病年龄较晚。增大的病变大小和绒毛变化似乎促进了腺瘤的进展,而不是息肉数量或不典型增生。也许是时候考虑一种新的MAP患者分期系统,以更好地确定疾病严重程度和监测策略。
BACKGROUND & AIMS: MUTYH-associated polyposis (MAP) is similar to familial adenomatous polyposis (FAP), in that it increases the risk for duodenal adenomas and cancer. Almost all patients with FAP develop duodenal adenomas and 5% develop duodenal cancer. Little is known about the prevalence of duodenal adenomas and cancer in patients with MAP, but current surveillance recommendations are the same for patients with FAP-they should begin surveillance when they are 25 years old. We aimed to assess the prevalence, extent, and progression of duodenal adenomas in patients with MAP and evaluate upper gastrointestinal tract surveillance recommendations.METHODS: In a retrospective study, we collected data on all patients (n = 92) with MAP undergoing surveillance esophagogastroduodenoscopy from registries at St Mark's Hospital (London, UK) and the Academic Medical Center (Amsterdam, The Netherlands) from 2002 through 2014. We collected information on adenoma development, age at adenoma detection, interventions, and disease progression.RESULTS: Duodenal adenomas were detected in 31 patients (34%), at a median age of 50 years. When duodenal polyposis first was detected, it was Spigelman stages I or II in 84% of patients; most had few small polyps, without high-grade dysplasia or villous features. Subsequent esophagogastroduodenoscopy evaluation of 18 of these patients found that 14 (78%) had Spigelman stages 0 to II disease (median follow-up period, 7.8 y). Disease progressed in stage in 6 patients, over 9.5 years, because of lesion size or villous features (2 reached stage IV disease). Adenomas were down-staged in 8 patients after biopsy or polypectomy analyses, and were unchanged for 3 patients.CONCLUSIONS: In a data analysis from 92 patients with MAP, duodenal polyposis seemed to develop less frequently than in patients with FAP, and developed at a later age. Increasing lesion size and villous change appear to promote adenoma progression, rather than polyp number or dysplasia. It may be time to consider a new staging system for patients with MAP, to better determine disease severity and surveillance strategies.