家族性腺瘤多发症（FAP）是一种罕见的遗传癌症易感综合征。FAP相关性球部病变（AL）的治疗具有挑战性，内窥镜括约肌切开术（EP）的作用尚未阐明。有限的FAP相关AL数据显示，至少在一定程度上结果不一致。我们回顾性分析了FAP相关和散发性球部病变（SAL）配对队列中EP的结果。ESAP研究包括1422个EP。进行了倾向得分匹配（最近邻法），包括年龄、性别、合并症、组织学亚型和大小。主要结果为完全切除（R0）、技术成功、并发症和复发。倾向得分匹配确定了202名患者（101名FAP，101名SAL），其基线特征相似。FAP患者主要无症状（79.2％[95％CI 71.2-87.3] vs. 46.5％[95％CI 36.6-56.4]，p <0.001）。初步R0率在FAP患者中明显较低（63.4％[95％CI 53.8-72.9] vs. 83.2％[95％CI 75.8-90.6]，p = 0.001）。经过反复介入后（每患者平均1.30次），R0相当（FAP 93.1％[95％CI 88.0-98.1] vs. SAL 97.0％[95％CI 93.7-100]，p = 0.194）。总体并发症率为28.7％。胰腺炎和出血是两组中最常见的不良事件。严重的并发症非常罕见（3.5％）。FAP组中21名患者（20.8％[95％CI 12.7-28.8]）和SAL组中16名患者（15.8％[95％CI 8.6-23.1]，p = 0.363）出现了复发。 FAP患者的复发发生时间较晚（25 [95％CI 18.3-31.7] vs. 2 [95％CI CI 0.06-3.9]个月）。EP对FAP相关性球部病变是安全和有效的。AL的内窥镜切除标准可以扩展到FAP患者。即使完全切除，FAP患者仍有终身复发风险，并需要长期监测。 Thieme保留所有权利。

Familial-adenomatous-polyposis (FAP) is a rare inherited cancer predisposition syndrome. The treatment for FAP-related ampullary lesions (AL) is challenging and the role of endoscopic papillectomy (EP) is not elucidated yet. Data of FAP associated AL are limited and showed, at least in part, inconclusive results. We retrospectively analyzed the outcomes of EP in matched cohorts of FAP-related and sporadic ampullary lesions (SAL).The ESAP study included 1422 EPs. A propensity-score matching (nearest-neighbor-method) including age, gender, comorbidity, histologic subtype and size was performed. Main outcomes were complete resection (R0), technical success, complications and recurrence.Propensity-score-based matching identified 202 patients (101 FAP, 101 SAL) with comparable baseline characteristics. FAP-patients were mainly asymptomatic (79.2% [95%CI 71.2-87.3] vs. 46.5% [95%CI 36.6-56.4]), p<0.001). The initial R0-rate was significantly lower in FAP-patients (63.4% [95%CI 53.8-72.9] vs. 83.2% [95%CI 75.8-90.6], p=0.001). After repeated (mean: 1.30 per patient) interventions, R0 was comparable (FAP 93.1% [95%CI 88.0-98.1] vs. SAL 97.0% [95%CI 93.7-100], p=0.194). The overall complication rate was 28.7%. Pancreatitis and bleeding were most common adverse events in both groups. Severe complications were very rare (3.5%). Twenty-one patients in the FAP-group (20.8% [95%CI 12.7-28.8]) and sixteen patients in the SAL-group (15.8% [95%CI 8.6-23.1], p=0.363) had a reccurence . Recurrences occurred later in FAP-patients (25 [95%CI 18.3-31.7] vs. 2 [95%CI CI 0.06-3.9] months).EP is safe and effective in FAP-related ampullary lesions Criteria for endoscopic resection of AL can be extended to FAP-patients. FAP-patients have a life-time risk to relapse even after complete resection and require long-time-surveillance.Thieme. All rights reserved.