Risk Factors and Causes of Death in MEN1 Disease. A GTE (Groupe d'Etude des Tumeurs Endocrines) Cohort Study Among 758 Patients
Risk Factors and Causes of Death in MEN1 Disease. A GTE (Groupe d'Etude des Tumeurs Endocrines) Cohort Study Among 758 Patients
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DOI：
10.1007/s00268-009-0290-1
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发表时间：
2010-02-01
影响因子：
2.6
通讯作者：
Bonithon-Kopp, Claire
中科院分区：
文献类型：
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作者：
Goudet, Pierre;Murat, Arnaud;Bonithon-Kopp, Claire
多发性内分泌瘤1型（MEN 1）的自然史是通过单机构或单家族研究而了解的。我们的目的是分析一个大型MEN 1患者队列的危险因素和死亡原因。通过GTE网络（Groupe d '，tude des Tumeurs Endocrines），共确定了758名有症状的MEN 1患者，该网络包括负责MEN 1诊断的法国和比利时遗传学实验室以及80个临床参考中心。分析死亡原因。一个包括时间依赖变量的虚弱模型被用来评估除甲状旁腺功能亢进以外的每种临床病变对生存率的影响，中位随访时间为6.3年。女性、MEN 1家族史和近期诊断与死亡风险较低相关。与未受影响的患者相比，胸腺肿瘤患者（风险比[HR] = 4.64，95% CI = 1.73-12.41），胰高血糖素瘤-血管紧张素瘤-生长抑素瘤（HR = 4.29，95% CI = 1.54-11.93），无功能性胰腺肿瘤经年龄、性别和诊断时间调整后，胃泌素瘤（HR = 3.43，95%CI = 1.71-6.88）和胃泌素瘤（HR = 1.89，95%CI = 1.09-3.25）的死亡风险较高。肾上腺肿瘤患者的死亡风险增加并不显著，但有3例患者死于侵袭性肾上腺肿瘤。胰腺癌、胰岛素瘤和支气管肿瘤并不增加死亡风险。1990年后MEN 1相关死亡比例从76.8%下降到71.4%。1980年以来MEN 1疾病的预后有所改善。胸腺肿瘤和胰腺肿瘤，包括非分泌性胰腺肿瘤，增加死亡风险。罕见但侵袭性的肾上腺肿瘤也可能导致死亡。大多数死亡与MEN 1有关。需要对腹部和胸部成像提出新的建议。
The natural history of multiple endocrine neoplasia type 1 (MEN1) is known through single-institution or single-family studies. We aimed to analyze the risk factors and causes of death in a large cohort of MEN1 patients.Overall, 758 symptomatic MEN1 patients were identified through the GTE network (Groupe d',tude des Tumeurs Endocrines), which involves French and Belgian genetics laboratories responsible for MEN1 diagnosis and 80 clinical reference centers. The causes of death were analyzed. A frailty model, including time-dependent variables, was used to assess the impact of each clinical lesion, except for hyperparathyroidism, on survival.The median follow-up was 6.3 years. Female gender, family history of MEN1, and recent diagnosis were associated with a lower risk of death. Compared with nonaffected patients, those with thymic tumors (hazard ratio [HR] = 4.64, 95% CI = 1.73-12.41), glucagonomas-vipomas-somatostatinomas (HR = 4.29, 95% CI = 1.54-11.93), nonfunctioning pancreatic tumors (HR = 3.43, 95% CI = 1.71-6.88), and gastrinoma (HR = 1.89, 95% CI = 1.09-3.25) had a higher risk of death after adjustment for age, gender, and diagnosis period. The increased risk of death among patients with adrenal tumors was not significant, but three patients died from aggressive adrenal tumors. Pituitary tumors, insulinomas, and bronchial tumors did not increase the risk of death. The proportion of MEN1-related deaths decreased from 76.8 to 71.4% after 1990.The prognosis of MEN1 disease has improved since 1980. Thymic tumors and duodenopancreatic tumors, including nonsecreting pancreatic tumors, increased the risk of death. Rare but aggressive adrenal tumors may also cause death. Most deaths were related to MEN1. New recommendations on abdominal and thoracic imaging are required.