协和医学杂志

2020, v.11(04) 365-369

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多发内分泌肿瘤治疗共识与争议
Consensus and Controversy on the Treatment of Multiple Endocrine Neoplasia

童安莉;李汉忠;
TONG An-li;LI Han-zhong;Department of Endocrinology, Key Laboratory of Endocrinology, National Health Commission of the People's Republic of China, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences &Peking Union Medical College;Department of Urology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences &Peking Union Medical College;

摘要(Abstract):

多发内分泌肿瘤(multiple endocrine neoplasia,MEN)是一种累及多种内分泌腺体的常染色体显性遗传病,常同时或先后发生两种以上内分泌肿瘤。MEN相关肿瘤包括原发性甲状旁腺功能亢进症、嗜铬细胞瘤、甲状腺髓样癌、胰腺神经内分泌肿瘤和垂体瘤等,这些肿瘤的临床特点和治疗方案与散发性肿瘤比较既有相似之处,亦有其特殊性。近年来,MEN相关肿瘤的治疗已经达成了一些共识,但仍存在较多争议,手术时机及手术切除范围是争议的焦点。
Multiple endocrine neoplasia(MEN) is an autosomal dominant hereditary disease involving multiple endocrine glands with two or more endocrine tumors occurring simultaneously or successively. MEN-related tumors include primary hyperparathyroidism, pheochromocytoma, medullary thyroid cancer, pancreatic neuroendocrine neoplasia, and pituitary adenoma, etc. Although the clinical characteristics and treatment of these tumors are similar to those of sporadic tumors, there are differences in some aspects. In recent years, there has been some consensus achieved on the treatment of MEN-related tumors. But there are also some controversies. The timing and scope of surgical resection are the focus of controversies.

关键词(KeyWords): 多发内分泌肿瘤;治疗;手术时机
multiple endocrine neoplasia;treatment;surgical timing

Abstract:

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基金项目(Foundation): 中国医学科学院医学与健康科技创新工程(2017-12M-1-001)

作者(Author): 童安莉;李汉忠;
TONG An-li;LI Han-zhong;Department of Endocrinology, Key Laboratory of Endocrinology, National Health Commission of the People's Republic of China, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences &Peking Union Medical College;Department of Urology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences &Peking Union Medical College;

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