[II B型多发性内分泌腺瘤病]

[Multiple endocrine neoplasia of the II B type].

作者信息

Schickedanz H, Hesse V, Kauf E, Mohr F

机构信息

Abteilung für Kinderchirurgie der Klinik und Poliklinik für Chirurgie des Bereiches Medizin der Friedrich-Schiller-Universität Jena, DDR.

出版信息

Z Kinderchir. 1989 Jun;44(3):181-5. doi: 10.1055/s-2008-1043232.

DOI:10.1055/s-2008-1043232

PMID:2568720

Abstract

Multiple endocrine neoplasia, type II B (MEN II B) includes thyroid carcinoma, pheochromocytoma, ganglioneuromatosis and marfanoid habitus. Its short-term prognosis is determined by the tumour of the adrenal gland, whereas long-term prognosis is determined by the thyroid carcinoma. Often the well-defined but rare syndrome is diagnosed late. The MEN II B's therapy consists of thyroidectomy and adrenalectomy uni- or bilateral. The 5 year-rate of survival is 75%. Own observations are reported.

摘要

多发性内分泌腺瘤病II B型（MEN II B）包括甲状腺癌、嗜铬细胞瘤、神经节瘤病和类马凡体型。其短期预后取决于肾上腺肿瘤，而长期预后则由甲状腺癌决定。这种明确但罕见的综合征往往诊断较晚。MEN II B的治疗包括单侧或双侧甲状腺切除术和肾上腺切除术。5年生存率为75%。报告了自身观察结果。