Background and Aims

The management of ampullary adenomas in familial adenomatous polyposis (FAP) is challenging due to multiple adenomas in the duodenum, history of previous major abdominal surgery, and desmoid lesions. In this study, we aim to define the optimum management for ampullary adenomas, particularly in FAP.

Methods

This is a retrospective study of all patients with pathology-confirmed ampullary adenomas in M.D. Anderson Cancer Center from 2002 to 2018. Relevant data were extracted, including patient demographics, treatments, outcomes, and adverse events.

Results

There were 137 patients with ampullary adenomas who underwent 159 procedures; 95 of the 137 patients had FAP and were placed under close observation, 29 underwent endoscopic ampullectomy, 4 underwent surgical ampullectomy, and 31 underwent panreaticoduodenectomy (PD). In the close observation group, 12.6% progressed to advanced adenoma and subsequently underwent resection. There was no ampullary cancer detected in this group. The endoscopic ampullectomy group had a postprocedure adverse event rate of 10.2%. Eleven patients had residual/recurrent disease after endoscopic ampullectomy, 3 of whom needed surgery. Four patients underwent initial surgical ampullectomy and 2 subsequently needed PD. Patients who underwent PD had an adverse event rate of 29%. In this group, there were no cases of residual disease or recurrence.

Conclusions

The management of ampullary adenomas in FAP should be carefully considered for the best outcome. Although these patients can be managed by endoscopic ampullectomy, careful surveillance for recurrence should be followed along with prompt management of the recurrence when detected. Although PD provides a definitive treatment, it is limited by the patient’s comorbid conditions and high adverse event rates.

中文翻译：

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家族性腺瘤性息肉病综合征壶腹腺瘤的治疗：从三级癌症中心拥有16年的经验。

背景和目标

家族性腺瘤性息肉病（FAP）壶腹腺瘤的治疗具有挑战性，这是由于十二指肠中存在多个腺瘤，既往有重大腹部手术史以及类胶质瘤病变。在这项研究中，我们旨在定义壶腹腺瘤的最佳治疗方法，尤其是在FAP中。

方法

这是一项2002年至2018年在MD Anderson癌症中心对所有经病理证实的壶腹腺瘤患者的回顾性研究。提取了相关数据，包括患者的人口统计学，治疗，结果和不良事件。

结果

137例壶腹腺瘤患者接受了159次手术。137例患者中有95例接受了FAP并进行了密切观察，其中29例接受了内镜下壶腹切除术，4例接受了手术壶腹切除术，31例接受了全十二指肠切除术（PD）。在密切观察组中，有12.6％进展为晚期腺瘤，随后接受了切除术。该组中未检测到壶腹癌。内镜下壶腹部切除术组的术后不良事件发生率为10.2％。内窥镜下壶腹切除术后有11例残留/复发性疾病，其中3例需要手术治疗。4例患者接受了最初的手术壶腹切除术，2例随后需要PD。接受PD治疗的患者不良事件发生率为29％。在这一组中，没有残留疾病或复发的病例。

结论

FAP壶腹腺瘤的处理应仔细考虑，以达到最佳效果。尽管这些患者可以通过内镜下壶腹切除术进行治疗，但应仔细监测复发情况，并在发现复发后立即进行治疗。尽管PD提供了确定的治疗方法，但它受到患者合并症和高不良事件发生率的限制。