多发性内分泌腺瘤-1型的临床特征分析
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江苏省人民医院

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Analysis of clinical features of multiple endocrine neoplasia type-1
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    摘要:

    目的 探讨多发性内分泌腺瘤-1型(Multiple endocrine neoplasia type-1,MEN-1)的临床特征,分析其病例特点并总结经验,以期更好地认识该病。 方法 收集南京医科大学第一附属医院2010 年 1 月至 2022年6月10 例MEN-1患者的临床资料,包括人口统计学资料、实验室检查、肿瘤的组分和治疗方式等。并通过检索 PubMed 数据库, 统计 2010 年 1 月至 2022年6月关于MEN-1的 24 例个案报道中患者资料,进行回顾性分析。 结果 本研究共纳入34例 MEN-1患者,男性21例,女性13例,发病中位年龄为55岁(30-80岁),其中20例患者有家族史,10例患者发生转移,最常见的肿瘤组分是甲状旁腺腺瘤(29例,29%),胰腺神经内分泌肿瘤(30例,30%),垂体瘤(20例,20%),29例甲状旁腺腺瘤均进行了手术治疗,28例胰腺神经内分泌肿瘤进行了手术治疗,其中有2例术后行化学疗法,2例术后行生物制剂治疗,另外2例长期行质子泵抑制剂治疗,20例垂体瘤中,有5例进行了手术治疗,8例行药物治疗,7例随访观察。随访至2022年6月,34例MEN-1患者中只有1例患者死亡。 结论 MEN-1的临床表现形式多种多样,甲状旁腺肿瘤、胰腺神经内分泌肿瘤和垂体肿瘤是其最常见的三大临床表现,手术治疗是各肿瘤的主要治疗方式,其余治疗包括生物制剂、化学疗法等等。 关键词 多发性内分泌腺瘤-1型,临床特征,治疗方式

    Abstract:

    Objective To know the disease better by investigating the clinical features,analyzing characteristics of the cases and summarizing the experience of Multiple endocrine neoplasia type-1 (MEN-1). Methods Collect the clinical data of 10 patients with MEN-1 in the First Affiliated Hospital with Nanjing Medical University from January 2010 to June 2022,including demographic data, laboratory tests, tumor components and treatment methods and so on. The data of 24 cases of MEN-1 reported from January 2010 to June 2022 are retrospectively analyzed by searching PubMed database. Results A total of 34 MEN-1 patients are enrolled in this study, including 21 males and 13 females, with the onset median age of 55 years (30-80 years old). Among them, 20 patients have family medical history and 10 patients have metastasis. The most common tumor components are parathyroid adenoma (29 cases, 29%) , pancreatic neuroendocrine tumor (30 cases, 30%), pituitary adenoma (20 cases, 20%). 29 cases of parathyroid adenoma were treated surgically.28 cases of pancreatic neuroendocrine tumor were also treated surgically , including 2 cases of postoperative chemotherapy, 2 cases of postoperative biological agents for patients, and other 2 cases of long-term proton pump inhibitor therapy. Among 20 cases of pituitary adenoma, 5 cases were treated surgically, 8 patients were treated with drugs and 7 patients were followed up. Up to June 2022, only 1 patient died among 34 patients with MEN-1. Conclusion The clinical manifestations of MEN-1 are diverse. Parathyroid tumor, pancreatic neuroendocrine tumor and pituitary tumor are the three most common clinical manifestations of MEN-1. Surgical treatment is the main treatment methods of each tumor, and other therapies include biological agents, chemotherapy and so on. Key words Multiple endocrine neoplasia type 1(MEN-1),clinical characteristics,treatment

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  • 收稿日期:2022-09-22
  • 最后修改日期:2023-03-06
  • 录用日期:2023-07-09
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