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骨肌上皮肿瘤

Myoepithelial Tumors of Bone.

作者信息

Song Wangzhao, Flucke Uta, Suurmeijer Albert J H

机构信息

Department of Pathology and Medical Biology, University Medical Center Groningen, University of Groningen, PO Box 30.001, Groningen 9700RB, The Netherlands.

Department of Pathology, Nijmegen Medical Center, Radboud University, PO Box 9101, Nijmegen 6500HB, The Netherlands.

出版信息

Surg Pathol Clin. 2017 Sep;10(3):657-674. doi: 10.1016/j.path.2017.04.010. Epub 2017 Jun 29.

Abstract

Myoepithelial tumors (METs) of bone (BMETs) are a rare but distinct tumor entity. METs that are cytologically benign are termed myoepitheliomas; METs with malignant histologic features are called myoepithelial carcinomas. BMETs have a wide age range, may involve any part of the skeleton, and have a variable spindle cell and epithelioid morphology. Bone tumors to be considered in the differential diagnosis are discussed. Additional techniques are indispensable to correctly diagnose BMETs. By immunohistochemistry, BMETs often express cytokeratins and/or EMA together with S100, GFAP, or calponin. Half of BMETs harbor EWSR1 (or rare FUS) gene rearrangements with different gene partners.

摘要

骨肌上皮肿瘤(BMETs)是一种罕见但独特的肿瘤实体。细胞学上为良性的肌上皮肿瘤称为肌上皮瘤;具有恶性组织学特征的肌上皮肿瘤称为肌上皮癌。BMETs的发病年龄范围广泛,可累及骨骼的任何部位,具有可变的梭形细胞和上皮样形态。文中讨论了鉴别诊断时需要考虑的骨肿瘤。正确诊断BMETs离不开其他技术。通过免疫组织化学,BMETs通常同时表达细胞角蛋白和/或EMA以及S100、GFAP或钙调蛋白。一半的BMETs存在EWSR1(或罕见的FUS)基因重排,且基因伴侣不同。

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