ERG expression in epithelioid sarcoma: a diagnostic pitfall.
ERG expression in epithelioid sarcoma: a diagnostic pitfall.
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上皮肉瘤中的ERG表达：诊断性陷阱。
DOI：
10.1097/pas.0b013e31828de23a
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发表时间：
2013-10
期刊：
影响因子：
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通讯作者：
Fetsch JF
中科院分区：
文献类型：
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作者：
Miettinen M;Wang Z;Sarlomo-Rikala M;Abdullaev Z;Pack SD;Fetsch JF
ERG转录因子在内皮细胞中组成性表达。由于良性和恶性血管内皮保留了ERG的表达，ERG被认为是血管肉瘤和相关肿瘤的有用标志物。由于涉及ERG易位，ERG也在前列腺癌和尤因肉瘤的一个亚群中表达，因此该标记物对这些恶性肿瘤的研究也有很高的兴趣。在这项研究中，我们基于对一个ERG阳性病例的初步观察，评估了109个上皮样肉瘤的ERG表达。我们还研究了其他内皮抗原在上皮样肉瘤中的表达。38%的上皮样肉瘤（41/109）表达ERG，通常呈均匀核染色，与血管肉瘤相似。然而，所有上皮样肉瘤的ERG基因重排均为阴性，这表明ERG的表达与上皮样肉瘤中涉及易位的ERG不太可能相关。其他内皮标志物CD31、claudin5和Prox1在上皮样肉瘤中不存在。唯一的例外是上皮样肉瘤的肺转移显示局灶性CD31表达，这可能是由于抗原吸附在肿瘤细胞表面造成的。然而，podoplanin在上皮样肉瘤中普遍表达（7/9），因此该标记物不能用于区分上皮样肉瘤和血管肉瘤。INI1/SMARCB1基因产物在所有上皮样肉瘤中均不存在（这里被认为是一个定义特征），但仅在一例上皮样血管肉瘤中不存在，表明其在鉴别诊断中对上皮样肉瘤的相对特异性。ERG表达在上皮样肉瘤中相当普遍，在上皮样肉瘤和上皮样血管肉瘤鉴别诊断中应被视为诊断缺陷。上皮样肉瘤普遍缺乏内皮细胞特异性标记物，有助于这种区分。
ERG transcription factor is constitutively expressed in endothelial cells. Because benign and malignant vascular endothelia retain the ERG-expression, ERG is considered a useful marker for angiosarcomas and related tumors. ERG is also expressed in a subset of prostate carcinomas and Ewing sarcomas due to ERG-involving translocations, so that this marker is also of high interest for the study of these malignancies. In this study, we evaluated 109 epithelioid sarcomas for ERG expression, based on an initial observation of an ERG-positive case. We also studied expression of other endothelial antigens in epithelioid sarcoma. ERG was expressed in 38% of epithelioid sarcomas (41/109), usually with a uniform nuclear staining, similar to that seen in angiosarcomas. However, all epithelioid sarcomas were negative for ERG gene rearrangement indicating that that ERG expression is not likely related to ERG involving translocations in epithelioid sarcoma. Other endothelial markers, CD31, claudin 5, and Prox1 were absent in epithelioid sarcomas. The only exception was a pulmonary metastasis of epithelioid sarcoma showing focal CD31 expression, which was probably resulting from antigen adsorption onto tumor cell surfaces. However, podoplanin was commonly (7/9) expressed in epithelioid sarcoma, so that this marker is not useful in the distinction of epithelioid sarcoma and angiosarcoma. INI1/SMARCB1 gene product was absent in all epithelioid sarcomas (considered here a definitional feature) but was absent from only one epithelioid angiosarcoma, indicating its relative specificity for epithelioid sarcoma in this differential diagnostic setting. ERG expression is fairly common in epithelioid sarcoma, and should be recognized as a diagnostic pitfall in the differential diagnosis of epithelioid sarcoma and epithelioid angiosarcoma. General lack of endothelial cell specific markers in epithelioid sarcoma helps in this distinction.