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手掌部肌上皮瘤样透明变性上皮样肿瘤:一例报告

Myoepithelioma-like hyalinizing epithelioid tumors of the palm: A case report.

作者信息

Bao Xiaoxiao, Lu Qianqian, Huang Bifei, Zhang Xiaowei

机构信息

Department of Pathology, Affiliated Dongyang Hospital of Wenzhou Medical University, Dongyang, Zhejiang, P.R. China.

出版信息

J Int Med Res. 2025 Mar;53(3):3000605251324492. doi: 10.1177/03000605251324492. Epub 2025 Mar 13.

Abstract

Myoepithelioma-like hyalinizing epithelioid tumors (MHETs) are distinct soft tissue neoplasms characterized by their unique histological presentation, including myoepithelioma-like hyalinization and an epithelioid-cell pattern. A defining molecular characteristic of these tumors is the presence of a novel :: gene fusion. Their distinct morphology makes diagnosis challenging because they can be mistaken for other soft tissue tumors. We report the case of a 71-year-old man who was admitted to our hospital with a 1-year history of a palpable mass on the left palm. Physical examination revealed a well-defined, mobile mass on the lateral aspect of the left palm, measuring 2.0 × 3.0 cm, with moderate consistency. The tumor was surgically excised, and postoperative pathology based on immunohistochemical staining and tissue morphology confirmed the diagnosis of MHET. The tumor cells exhibited diffuse positivity for CD34 and focal positivity for EMA. At the 3-month follow-up post-surgery, there was no evidence of recurrence or distant metastasis. MHETs are typically observed as superficial, well-circumscribed masses without the histological features of malignancy. Given the distinct tissue morphology and the recent identification of this tumor type, clinicians and pathologists must be aware of these features to facilitate accurate diagnosis and inform treatment strategies.

摘要

肌上皮瘤样透明变性上皮样肿瘤(MHETs)是一种独特的软组织肿瘤,其特征在于独特的组织学表现,包括肌上皮瘤样透明变性和上皮样细胞模式。这些肿瘤的一个决定性分子特征是存在一种新的::基因融合。它们独特的形态使诊断具有挑战性,因为它们可能被误诊为其他软组织肿瘤。我们报告一例71岁男性患者,因左手掌可触及肿块1年入院。体格检查发现左手掌外侧有一个边界清晰、可活动的肿块,大小为2.0×3.0 cm,质地中等。肿瘤经手术切除,术后基于免疫组织化学染色和组织形态学的病理检查确诊为MHET。肿瘤细胞CD34呈弥漫性阳性,EMA呈局灶性阳性。术后3个月随访时,无复发或远处转移迹象。MHETs通常表现为浅表、边界清晰的肿块,无恶性组织学特征。鉴于这种肿瘤类型独特的组织形态以及最近才被识别,临床医生和病理学家必须了解这些特征,以便准确诊断并为治疗策略提供依据。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/285e/11907517/0673116f7ad1/10.1177_03000605251324492-fig1.jpg

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