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首页> 外文期刊>Journal of Medical Case Reports >Primary vaginal Ewing's sarcoma or primitive neuroectodermal tumor in a 17-year-old woman: a case report
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Primary vaginal Ewing's sarcoma or primitive neuroectodermal tumor in a 17-year-old woman: a case report

机译：一名17岁妇女的原发性阴道尤文氏肉瘤或原始神经外胚层肿瘤：一例病例报告

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Introduction Primary Ewing's sarcoma or primitive neuroectodermal tumor of the genital tract of women is uncommon. Rarer still is its occurrence in the vagina, with only five cases described so far. Out of these, only one case was confirmed using molecular analysis. Case presentation We present an extremely rare case of Ewing's sarcoma or primitive neuroectodermal tumor in a 17-year-old Indian girl. She presented with a vaginal mass that was initially diagnosed as a malignant round cell tumor. Immunohistochemistry showed diffuse positivity for vimentin, membranous positivity for MIC2, and positivity for BCL2 and FLI-1. On the other hand, she was negative for cytokeratin, epithelial membrane antigen, desmin, Myo D-1, myogenin and smooth muscle actin. A diagnosis of primitive neuroectodermal tumor was thus offered. Furthermore, a molecular analysis of our patient using reverse transcription-polymerase chain reaction technique showed positivity for t(11; 22) (q24; q12) (EWSR1-FLI1), thus confirming the diagnosis of a Ewing's sarcoma/primitive neuroectodermal tumor. Our patient was offered chemotherapy on Institutional protocol EFT 2001. Conclusion This is a rare case of primary vaginal Ewing's sarcoma or primitive neuroectodermal tumor, which was confirmed with molecular analysis, in the youngest patient known so far. This study reinforces the value of integrating morphological features with membranous MIC2 positivity, along with application of molecular techniques in objective identification of an Ewing's sarcoma or primitive neuroectodermal tumor at uncommon sites.
机译：引言女性原发性尤文氏肉瘤或原始生殖器神经外胚层肿瘤并不常见。它在阴道中的发生仍然很少，到目前为止仅描述了五例。其中，仅通过分子分析确认了1例。病例介绍我们介绍了一个17岁的印度女孩中尤因肉瘤或原始神经外胚层肿瘤的极为罕见的病例。她表现出阴道肿块，最初被诊断为恶性圆形细胞瘤。免疫组织化学显示波形蛋白的弥散阳性，MIC2的膜阳性，BCL2和FLI-1的阳性。另一方面，她的细胞角蛋白，上皮膜抗原，结蛋白，Myo D-1，肌生成素和平滑肌肌动蛋白阴性。因此提供了对原始神经外胚层肿瘤的诊断。此外，使用逆转录-聚合酶链反应技术对我们的患者进行的分子分析显示，t（11; 22）（q24; q12）（EWSR1-FLI1）呈阳性，从而证实了尤文氏肉瘤/原始神经外胚层肿瘤的诊断。我们的患者在2001年EFT机构规程下接受了化学疗法的治疗。结论这是迄今为止已知的最年轻患者中罕见的原发性阴道尤文氏肉瘤或原始神经外胚层肿瘤病例，经分子分析证实。这项研究强调了将形态特征与膜性MIC2阳性相结合的价值，以及分子技术在罕见部位尤文氏肉瘤或原始神经外胚层肿瘤的客观鉴定中的应用。

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