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[肝脏原发性血管周上皮样细胞瘤(PEComa)——肝脏肿瘤的一种新类型?]

[Primary perivascular epitheloid cell tumour (PEComa) of the liver - is a new entity of the liver tumors?].

作者信息

Panahova S, Rempp H, Sipos B, Malek N P, Boozari B

机构信息

Medizinische Klinik I, Universitätsklinikum Tübingen, Tübingen, Germany.

Abteilung für diagnostische und interventionelle Radiologie, Radiologische Klinik, Eberhard-Karls-Universität Tübingen, Tübingen, Germany.

出版信息

Z Gastroenterol. 2015 May;53(5):399-408. doi: 10.1055/s-0034-1399391. Epub 2015 May 12.

Abstract

Perivascular epitheloid cell tumor (PEComa) is a rare tumor, characterized by dual Expression of smooth muscle and melanocytic markers. Due to the development of diagnostic procedures, we now diagnose PEComa more often. We report about a case of PEComa of the liver as an accidental finding. We analyze the clinical and morphological characteristics of this tumor and compare it with the data of the literature. Management of patients with PEComa is not yet standardized; therefore biopsy with immunhistochemical staining is necessary for the diagnosis. In case of liver tumors which cannot be classified by their morphology on imaging modalities, it is important to think about this rare entity.

摘要

血管周上皮样细胞肿瘤(PEComa)是一种罕见肿瘤,其特征是平滑肌和黑素细胞标志物双重表达。由于诊断程序的发展,我们现在更常诊断出PEComa。我们报告一例肝脏PEComa作为偶然发现的病例。我们分析了该肿瘤的临床和形态学特征,并与文献数据进行了比较。PEComa患者的管理尚未标准化;因此,活检及免疫组织化学染色对于诊断是必要的。对于在影像学上无法根据形态学分类的肝脏肿瘤,考虑这种罕见实体很重要。

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