Expression of PAX3 in Ewing's Sarcoma Family of Tumors

Theodor W. Schulte , Jeffrey A. Toretsky , Elisabeth Ress , Lee Helman , Leonard M. Neckers
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引用次数: 33

Abstract

The Ewing's sarcoma family of tumors (ESFT) is the second most common pediatric malignancy originating in the bone and is characterized by the t(11; 22) translocation. PAX3, a member of the paired box family of genes, is expressed during embryonal development of neural crest cells and is involved in the t(2; 13) translocation found in alveolar rhabdomyosarcoma. Since ESFTs are believed to be derived from neural crest tissue, we screened a series of Ewing's sarcoma and peripheral neuroectodermal tumor cell lines and tumor specimens for expression of PAX3. We found expression of PAX3 in most, but not all, of the specimens analyzed, including cell lines and patient material.

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PAX3在Ewing肉瘤家族肿瘤中的表达
尤文氏肉瘤家族肿瘤(ESFT)是第二常见的儿童恶性肿瘤,起源于骨骼,其特征是t(11;22)易位。PAX3是配对盒家族基因的一员,在神经嵴细胞胚胎发育过程中表达,参与t(2;13)肺泡横纹肌肉瘤易位。由于ESFTs被认为来源于神经嵴组织,我们筛选了一系列尤文氏肉瘤和周围神经外胚层肿瘤细胞系和肿瘤标本,检测PAX3的表达。我们在大多数(但不是全部)分析的标本中发现了PAX3的表达,包括细胞系和患者材料。
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