Long-term outcomes in patients with multiple endocrine neoplasia type 1 and pancreaticoduodenal neuroendocrine tumours
Long-term outcomes in patients with multiple endocrine neoplasia type 1 and pancreaticoduodenal neuroendocrine tumours
复制标题
DOI：
10.1111/cen.13264
复制
发表时间：
2017-02-01
影响因子：
3.2
通讯作者：
Young, W. F., Jr.
中科院分区：
文献类型：
--
作者：
Donegan, D.;Ospina, N. Singh;Young, W. F., Jr.
背景在多发性内分泌肿瘤 1 型 (MEN-1) 患者中，胰十二指肠 (PD) 神经内分泌肿瘤 (NET) 与早期死亡率相关，但最佳治疗策略仍不确定。目的评估 PD-NET 患者的重要结局（死亡率和转移）以及 MEN-1 患者结局的预测因素。方法对来自明尼苏达州罗切斯特市梅奥诊所的 MEN-1 患者进行回顾性队列研究1997 年至 2014 年。结果我们确定了 287 名 MEN-1 患者； 199 名 (69%) 患者有 217 个 PD-NET。在患有 PD-NET 的患者中，129 人 (65%) 接受了手术，其中 90 人 (70%) 在 Mayo Clinic 进行了初次手术。术后中位随访时间为 8 年，期间 13 名患者 (14%) 死亡。平均（标准差）死亡年龄为 51 (+/- 9) 岁。肿瘤大小、手术时的转移或肿瘤类型并不能预测死亡率，但手术时年龄每增加一岁，转移的几率就会增加 6%。 70 名 (35%) 患者未进行手术。在那些没有已知转移性疾病的观察/医疗治疗患者中，平均肿瘤生长为 002 厘米/年（范围，-013-04 厘米/年）。 4 名患者 (7%) 的中位年龄为 77 岁（范围为 51-89）岁。结论 PD-NET 在 MEN-1 患者中很常见，即使在手术干预后也与早期死亡相关。尽管缺乏识别此类患者的明确因素，但主动监测是非攻击性 PD-NET 的可行选择。因此，有关当前治疗方案的风险和益处的咨询仍然是 MEN-1 患者护理中不可或缺的一部分。
BackgroundIn patients with multiple endocrine neoplasia type 1 (MEN-1), pancreaticoduodenal (PD) neuroendocrine tumours (NETs) are associated with early mortality, yet the best treatment strategy remains uncertain.AimTo assess patient important outcomes (mortality and metastasis) of PD-NETs and predictors of outcomes in patients with MEN-1.MethodsRetrospective cohort of patients with MEN-1 who attended the Mayo Clinic, Rochester, MN from 1997 to 2014.ResultsWe identified 287 patients with MEN-1; 199 (69%) patients had 217 PD-NETs. Among those with a PD-NETs, 129 (65%) had surgery of which 90 (70%) had their primary surgery performed at Mayo Clinic. The median postoperative follow-up was 8 years during which 13 (14%) patients died. The mean (standard deviation) age of death was 51 (+/- 9) years. Tumour size, metastasis at surgery or tumour type were not predictive of mortality, but for every year older at surgery, the odds of metastasis increased by 6%. Surgery was not performed in 70 (35%) patients. Among those who were observed/medically managed without known metastatic disease, mean tumour growth was 002 cm/year (range, -013-04 cm/year). Four patients (7%) died at a median age of 77 (range, 51-89) years.ConclusionPD-NETs are common in patients with MEN-1 and are associated with early mortality even after surgical intervention. Active surveillance is a viable option in nonaggressive PD-NETs, although definitive factors identifying such patients are lacking. Therefore, counselling regarding risks and benefits of current treatment options remains integral to the care of patients with MEN-1.