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Abstract：
Multiple endocrine neoplasia type 1 (MEN1)-related neuroendocrine tumors (NETs) of the lung are mostly indolent, with a good prognosis. Nevertheless, cases of aggressive lung NET do occur, and therefore the management of individual patients is challenging.
To assess tumor growth and the survival of patients with MEN1-related lung NETs at long-term follow-up.
The population-based Dutch MEN1 Study Group database (n = 446) was used to identify lung NETs by histopathological and radiological examinations. Tumor diameter was assessed. Linear mixed models and the Kaplan-Meier method were used for analyzing tumor growth and survival. Molecular analyses were performed on a lung NET showing particularly aggressive behavior.
In 102 patients (22.9% of the total MEN1 cohort), 164 lesions suspected of lung NETs were identified and followed for a median of 6.6 years. Tumor diameter increased 6.0% per year. The overall 15-year survival rate was 78.0% (95% confidence interval: 64.6-94.2%) without lung NET-related death. No prognostic factors for tumor growth or survival could be identified. A somatic c.3127A > G (p.Met1043Val) PIK3CA driver mutation was found in a case of rapid growing lung NET after 6 years of indolent disease, presumably explaining the sudden change in course.
MEN1-related lung NETs are slow growing and have a good prognosis. No accurate risk factors for tumor growth could be identified. Lung NET screening should therefore be based on well-informed, shared decision-making, balancing between the low absolute risk of an aggressive tumor in individuals and the potential harms of frequent thoracic imaging.
To assess tumor growth and the survival of patients with MEN1-related lung NETs at long-term follow-up.
The population-based Dutch MEN1 Study Group database (n = 446) was used to identify lung NETs by histopathological and radiological examinations. Tumor diameter was assessed. Linear mixed models and the Kaplan-Meier method were used for analyzing tumor growth and survival. Molecular analyses were performed on a lung NET showing particularly aggressive behavior.
In 102 patients (22.9% of the total MEN1 cohort), 164 lesions suspected of lung NETs were identified and followed for a median of 6.6 years. Tumor diameter increased 6.0% per year. The overall 15-year survival rate was 78.0% (95% confidence interval: 64.6-94.2%) without lung NET-related death. No prognostic factors for tumor growth or survival could be identified. A somatic c.3127A > G (p.Met1043Val) PIK3CA driver mutation was found in a case of rapid growing lung NET after 6 years of indolent disease, presumably explaining the sudden change in course.
MEN1-related lung NETs are slow growing and have a good prognosis. No accurate risk factors for tumor growth could be identified. Lung NET screening should therefore be based on well-informed, shared decision-making, balancing between the low absolute risk of an aggressive tumor in individuals and the potential harms of frequent thoracic imaging.
摘要：
多发性内分泌瘤1型（MEN1）相关的肺神经内分泌肿瘤（NETs）大多为惰性，预后良好。然而，确实发生侵袭性肺NET的病例，因此，个别患者的管理是具有挑战性的。
在长期随访中评估MEN1相关肺NETs患者的肿瘤生长和生存率。
基于人群的荷兰MEN1研究组数据库（n=446）用于通过组织病理学和放射学检查鉴定肺NETs。评估肿瘤直径。线性混合模型和Kaplan-Meier方法用于分析肿瘤生长和存活。对显示出特别侵袭性行为的肺NET进行分子分析。
在102名患者中（占MEN1队列总数的22.9%），我们发现了164个疑似肺部NETs的病灶,随访时间中位数为6.6年.肿瘤直径每年增加6.0%。总体15年生存率为78.0％（95％置信区间：64.6-94.2％），无肺部NET相关死亡。没有确定肿瘤生长或存活的预后因素。体细胞c.3127A>G（p。Met1043Val)PIK3CA驱动突变在持续6年的无痛性疾病后快速生长的肺部NET中发现，大概解释了过程中的突然变化。
MEN1相关肺NETs生长缓慢，预后良好。无法确定肿瘤生长的准确危险因素。因此，肺部网络筛查应基于消息灵通，共同决策，在个体中侵袭性肿瘤的低绝对风险与频繁胸部成像的潜在危害之间取得平衡。
在长期随访中评估MEN1相关肺NETs患者的肿瘤生长和生存率。
基于人群的荷兰MEN1研究组数据库（n=446）用于通过组织病理学和放射学检查鉴定肺NETs。评估肿瘤直径。线性混合模型和Kaplan-Meier方法用于分析肿瘤生长和存活。对显示出特别侵袭性行为的肺NET进行分子分析。
在102名患者中（占MEN1队列总数的22.9%），我们发现了164个疑似肺部NETs的病灶,随访时间中位数为6.6年.肿瘤直径每年增加6.0%。总体15年生存率为78.0％（95％置信区间：64.6-94.2％），无肺部NET相关死亡。没有确定肿瘤生长或存活的预后因素。体细胞c.3127A>G（p。Met1043Val)PIK3CA驱动突变在持续6年的无痛性疾病后快速生长的肺部NET中发现，大概解释了过程中的突然变化。
MEN1相关肺NETs生长缓慢，预后良好。无法确定肿瘤生长的准确危险因素。因此，肺部网络筛查应基于消息灵通，共同决策，在个体中侵袭性肿瘤的低绝对风险与频繁胸部成像的潜在危害之间取得平衡。
