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皮肤合体性肌上皮瘤:一种具有严重诊断陷阱的难以描述的皮肤肿瘤。

Cutaneous Syncytial Myoepithelioma: A Nondescript Skin Tumor With Serious Diagnostic Pitfalls.

作者信息

Wales Cameron, Diamond Scott, Hinds Brian

机构信息

UC San Diego Health, San Diego, CA, USA.

出版信息

Int J Surg Pathol. 2020 Feb;28(1):63-67. doi: 10.1177/1066896919879755. Epub 2019 Oct 6.

Abstract

Cutaneous syncytial myoepithelioma (CSM) is a rare tumor with a nondescript clinical presentation in the skin. It represents a relatively uncommon diagnostic entity with approximately 50 examples in the literature to date. We present a 36-year-old man with a new, tender 3-mm firm pink papule on the left bicep, in which a superficial shave technique produced a diagnostic challenge. CSM often poses a diagnostic quandary given shared histomorphological and immunohistochemical attributes with superficial mesenchymal or neurocristic tumors, namely, melanocytic proliferations, fibrous histiocytoma, and epithelioid sarcoma. The molecular profile of CSM may reveal or gene rearrangement, but as we showcase, the diagnosis remains possible in the absence of this oncogenic fusion. Ultimately, there are pitfalls to avoid to correctly distinguish this benign myoepithelial lesion from more biologically aggressive neoplasia.

摘要

皮肤合体性肌上皮瘤(CSM)是一种罕见的肿瘤,在皮肤上临床表现不典型。它是一种相对不常见的诊断实体,迄今为止文献中约有50例报道。我们报告一名36岁男性,左二头肌处出现一个新的、触痛的3毫米坚实粉红色丘疹,采用浅表剃除术进行诊断具有挑战性。鉴于CSM与浅表间叶性或神经嵴性肿瘤(即黑素细胞增生、纤维组织细胞瘤和上皮样肉瘤)具有共同的组织形态学和免疫组化特征,其常常造成诊断上的困境。CSM的分子特征可能显示ALK或TFG基因重排,但正如我们所展示的,在没有这种致癌性融合的情况下仍有可能做出诊断。最终,要正确区分这种良性肌上皮病变与更具生物学侵袭性的肿瘤,有一些陷阱需要避免。

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