Background and Aims

Ampullary adenomas (AAs), common in familial adenomatous polyposis (FAP), are precursors to ampullary carcinoma. We assessed the natural history of AAs and factors associated with clinically significant progression (CSP).

Methods

Consecutive FAP patients with AAs and at least 2 EGDs were identified from a hereditary GI cancer registry. We assessed the incidence of CSP (increase in size to ≥10 mm and/or development of advanced histology) of AAs. Clinical, endoscopic, and pathologic features between patients with CSP and nonprogressors were compared.

Results

One hundred forty-three patients with AAs were included. Over a median follow-up of 7.8 years (interquartile range, 4.3-11.1), 41 patients (28.6%) developed CSP for an incidence of 35 per 1000 patient-years. Of 143 patients, 22 (15.6%) progressed to AAs ≥10 mm, 12 (8.5%) progressed to advanced histology, and 7 (4.9%) progressed both in size and histology. Two patients (1.4%) developed ampullary cancer. Male gender, abnormal appearance of the papilla at initial AA detection, prior cholecystectomy, and personal history of extracolonic malignancy were associated with CSP. Neither Spigelman stage nor the adenomatous polyposis coli gene pathogenic variants were associated with CSP. An intervention specifically for AA and not duodenal polyposis was performed in 24% of patients with AAs, including endoscopic papillectomy in 23 patients and duodenectomy in 3 patients at a median observation of 8.2 years.

Conclusions

Most FAP patients with AAs did not experience CSP or require resection over 8 years of surveillance. Ampullary cancer was rare. Male gender, abnormal appearance of the papilla at AA detection, cholecystectomy, and history of extracolonic malignancy were associated with CSP. Our findings favor endoscopic surveillance of AAs over expedited resection for most patients with FAP.

中文翻译：

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家族性腺瘤性息肉病壶腹腺瘤的自然史：一项长期随访研究

背景和目标

壶腹腺瘤 (AAs) 常见于家族性腺瘤性息肉病 (FAP)，是壶腹癌的前兆。我们评估了 AA 的自然史和与临床显着进展 (CSP) 相关的因素。

方法

从遗传性胃肠道癌症登记中确定了连续患有 AA 和至少 2 次 EGD 的 FAP 患者。我们评估了 AA 的 CSP（增大至 ≥10 mm 和/或发展为晚期组织学）的发生率。比较了 CSP 患者和非进展者之间的临床、内镜和病理特征。

结果

包括 143 名 AA 患者。在中位随访 7.8 年（四分位距，4.3-11.1）中，41 名患者（28.6%）发生 CSP，发病率为 35/1000 患者年。在 143 名患者中，22 名 (15.6%) 进展为 AA ≥ 10 mm，12 名 (8.5%) 进展至晚期组织学，7 名 (4.9%) 患者在大小和组织学方面都有进展。两名患者（1.4%）发展为壶腹癌。男性、初始 AA 检测时乳头异常外观、既往胆囊切除术和结肠外恶性肿瘤的个人病史与 CSP 相关。Spigelman 阶段和腺瘤性结肠息肉病基因致病变异均与 CSP 无关。24% 的 AA 患者接受了专门针对 AA 而不是十二指肠息肉病的干预，

结论

大多数患有 AA 的 FAP 患者在 8 年的监测中没有经历过 CSP 或需要切除。壶腹癌很少见。男性、AA 检测时乳头异常外观、胆囊切除术和结肠外恶性肿瘤病史与 CSP 相关。对于大多数 FAP 患者，我们的研究结果有利于对 AA 进行内镜监测而不是快速切除。