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[多发性内分泌腺瘤病]

[Multiple endocrine neoplasia].

作者信息

Takami Hiroshi, Ikeda Yoshifumi, Kameyama Kaori

机构信息

Department of Surgery, Teikyo University School of Medicine, 2-11-1 Kaga, Itabashi-ku, Tokyo 173-8605, Japan.

出版信息

Gan To Kagaku Ryoho. 2002 Apr;29(4):508-11.

Abstract

In multiple endocrine neoplasia (MEN) syndromes, tumors appear either synchronously or heterochronously in a combination of 2 or more specified endocrine glands. They are broadly classified as MEN 1 or MEN 2 according to the combination of tumors, and are inherited as an autosomal dominant trait. The genes responsible for these syndromes have been identified, making early diagnosis based on familial screening possible. The gene responsible for MEN 1 is located on the long arm of chromosome 11. It is a tumor suppressor gene that codes for menin. The cause of MEN 2 type is a mutation of the RET proto-oncogene on the long arm of chromosome 10. The present article discusses the diagnosis and treatment of these syndromes.

摘要

在多发性内分泌肿瘤(MEN)综合征中,肿瘤在2个或更多特定内分泌腺中以同步或异时的方式出现。根据肿瘤的组合,它们大致分为MEN 1或MEN 2,并作为常染色体显性性状遗传。导致这些综合征的基因已被确定,这使得基于家族筛查的早期诊断成为可能。MEN 1相关基因位于11号染色体长臂上。它是一种肿瘤抑制基因,编码menin。MEN 2型的病因是10号染色体长臂上RET原癌基因的突变。本文讨论了这些综合征的诊断和治疗。

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