遗传综合征，如结节性硬化症（TSC），占胰腺神经内分泌肿瘤（PNETs）的10％。对于大小大于2 cm 的散发性PNETs（spPNETs），手术干预是目前的标准护理。我们比较了切除的TSC-PNETs患者与spPNETs患者的长期结果。我们回顾分析了TSC-PNETs与spPNETs的围手术期数据和结果。纳入标准是选择肿瘤大小不超过5.1 cm 的患者，这是TSC-PNET组观察到的最大大小。在347例PNETs切除患者中，有14例是TSC-PNETs，241例是非功能性spPNETs。整个队列的中位年龄为56岁（四分位间距[IQR] 21.0），其中47%为女性。中位随访时间为103.8个月（95％置信区间[CI] 89.2-118.6）。具体而言，纳入了14例TSC-PNETs患者和194例spPNETs患者。与spPNETs相比，TSC-PNETs患者的手术年龄较小（24.0 vs. 57.5岁；p < 0.001），多灶性病变较常见（28.5% vs. 0.0%；p < 0.001），更有可能进行微创手术（78.6% vs. 24.3%；p < 0.001），以及更多的R1切除（28.6% vs. 5.7%；p = 0.006）。只有spPNET组观察到局部和远处肿瘤复发。spPNET组的5年死亡率为6.2%，而TSC-PNET组为0.0%。TSC-PNET患者中没有观察到与PNET相关的死亡。在中位随访78.0个月后，TSC-PNET患者中没有复发。对于TSC-PNET患者进行激进的胰腺手术的风险和效益仍不清楚，我们的发现表明应考虑保守的方法。© 2023. 作者。

Hereditary syndromes such as tuberous sclerosis complex (TSC) account for 10% of pancreatic neuroendocrine tumors (PNETs). Surgical intervention is the current standard of care for sporadic PNETs (spPNETs) that are >2 cm in size. We compared the long-term outcomes of resected TSC-PNETs with patients with spPNETs.We conducted a retrospective review of perioperative data and outcomes of TSC-PNETs compared with spPNETs. Inclusion criteria involved selecting patients whose tumors were no larger than 5.1 cm, the maximum size observed in the TSC-PNET group.Of the 347 patients resected for PNETs, 14 were TSC-PNETs and 241 were non-functional spPNETs. The median age for the whole cohort was 56 years (interquartile range [IQR] 21.0) and 47% were female. The median follow-up was 103.8 months (95% confidence interval [CI] 89.2-118.6). Specifically, 14 patients with TSC-PNETs and 194 patients with spPNETs were included. Compared with spPNETs, patients with TSC-PNETs were operated on at a younger age (24.0 vs. 57.5 years; p < 0.001), were more frequently multifocal (28.5% vs. 0.0%; p < 0.001), were more likely to undergo minimally invasive operations (78.6% vs. 24.3%; p < 0.001), and had more R1 resections (28.6% vs. 5.7%; p = 0.006). Local and distant tumor recurrence was only observed in the spPNET group. The 5-year mortality rates for the spPNET and TSC-PNET groups were 6.2% and 0.0%, respectively. No PNET-related deaths were observed among TSC-PNETs.None of the TSC-PNET patients recurred after a median follow-up of 78.0 months. The risk-benefit of aggressive pancreatic operations in TSC-PNET patients is still unclear and our findings suggest a conservative approach should be considered.© 2023. The Author(s).