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多核巨人:揭示儿童脏表皮质瘤PEComa
Aileen Azari-Yam1, Mohammad Vasei2, Moeinadin Safavi1
1Division of Molecular Pathology and Cytogenetics, Pathology Department, Children's Medical Center, Faculty of Medicine, Tehran University of Medical Sciences, Tehran, Iran.
Fetal and pediatric pathology
|February 21, 2025
概括
这项研究报告了一例罕见的3岁女孩上皮周血管上皮细胞瘤 (PEComa) 病例. 通过超声检测发现的儿科性PEComa显示不确定的恶性潜力,但患者在手术后恢复良好.
科学领域:
- 在瘤学瘤学.
- 儿科病理学 儿科病理学
- 腎臟病學 (nephrology) 是一種醫學專業.
背景情况:
- 周周血管上皮质细胞瘤 (PEComas) 是一种罕见的介质细胞瘤.
- 脏的PEComas非常罕见,特别是在儿童群体中.
- 结核性硬化症 (TS) 与PEComa风险增加有关,但这种病例发生在没有TS的患者身上.
研究的目的:
- 报告儿科患者上皮质PEComa的罕见病例.
- 分析这种罕见实体的组织学特征和临床表现.
- 审查和促进对儿科脏PEComas的理解.
主要方法:
- 一个3岁女孩的病例报告,诊断出右上皮状瘤PEComa.
- 通过超声波成像来识别瘤.
- 根性切除样本的组织病理学检查,包括细胞形态和排列.
- 对儿科性PEComa病例的文献综述.
主要成果:
- 表皮状PEComa呈现出表皮状和状细胞的独特周围血管布局,丰富的eosinophilic或透明细胞质,以及多核巨细胞.
- 瘤表现出不确定的恶性潜力.
- 患者尽管没有结核性硬化病史,但在切除术后的十个月内恢复良好,没有进一步的治疗.
结论:
- 儿科上皮质皮质瘤很少见,并且可能发生在没有结核性硬化症的患者中.
- 组织病理学特征,包括多核巨细胞的存在,对于诊断很重要.
- 整体手术切除似乎是治疗未确定恶性潜力的儿科脏PEComa的有效方法.
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