阳性雌激素受体EWSR1:FEV-重新排列的前列腺尤文肉瘤模仿高度神经内分泌癌
Tanisha Martheswaran1, Ezra Baraban1,2, John Gross1,2
1Department of Pathology, Johns Hopkins School of Medicine, Baltimore, MD, USA.
International journal of surgical pathology
|February 3, 2026
概括
这项研究报告了前列腺中尤宁肉瘤 (ES) 的极为罕见病例,通过EWSR1::FEV融合证实了这一情况. 这一发现突出了由于其模仿神经内分泌癌的诊断挑战.
科学领域:
- 在瘤学瘤学.
- 分子病理学分子病理学
- 尿道瘤学 尿道瘤学
背景情况:
- 尤文瘤 (ES) 是一种罕见的,激进的瘤,主要影响儿童和青少年,并很少出现骨外发生.
- 以EWSR1::FEV转位为特征的ES异常罕见,经常呈现出多样化的形态,免疫类型和攻击性行为,通常在骨外部位.
研究的目的:
- 介绍一个独特的前列腺ES病例与EWSR1::FEV融合.
- 详细介绍这种罕见瘤的临床,组织病理学,免疫类型和分子特征.
- 讨论前列腺ES的诊断考虑和管理策略.
主要方法:
- 一个患有前列腺质量患者的病例报告.
- 组织病理学检查和免疫组织化学 (IHC) 包括角质素,染色素,合成素,NKX2.2,CD99,PSA,NKX3.1和Ki67.
- 下一代测序 (NGS) 用于分子分析.
- 对雄激素受体 (AR) 表达的分析.
主要成果:
- 一个中年男子的前列腺质块显示出差分的上皮质瘤与神经内分泌标记物的特征,最初模仿神经内分泌癌.
- 免疫组织化学揭示了细胞激素,神经内分泌标记物,NKX2.2和CD99的表达,焦点NKX3.1阳性和负PSA.
- NGS证实了EWSR1::FEV融合,确立了ES的诊断. 瘤显示出强烈的AR阳性,表明前列腺起源.
结论:
- 前列腺ES与EWSR1::FEV融合是一种极其罕见的实体,由于重叠的免疫类型特征,可以被误认为是神经内分泌癌.
- 准确的诊断需要组织病理学,IHC和分子测试的结合,特别是NGS用于融合检测.
- 在这种情况下,强烈的AR阳性表明抗雄激素剥夺疗法的潜在效用,需要在类似的罕见恶性瘤中进行进一步的研究.
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