皮肤上皮质神经纤维瘤在脸上发生:病例报告和文献综述
Ihar I Haiduk1, Andrey Y Valkov1,2
1UNIM LLC, Moscow, Russia; and.
The American Journal of dermatopathology
|January 22, 2026
概括
我们呈现了一个罕见的高度表皮质肌纤维素瘤在脸上的罕见病例. 这种诊断需要采用多模式的方法,因为其具有挑战性的呈现和模仿其他恶性瘤.
科学领域:
- 在瘤学瘤学.
- 皮肤病理学 皮肤病理学
- 手术病理学手术病理学
背景情况:
- 肌肉纤维瘤是一种多态恶性瘤.
- 表皮状变体是罕见的,可以模仿非介质瘤.
- 皮肤瘤,尤其是面部瘤,会给诊断带来挑战.
研究的目的:
- 报告一种异常罕见的皮肤表皮质肌纤维素瘤病例.
- 突出诊断困难和必要的多式联运方法.
- 描述这种罕见实体的组织病理学和分子特征.
主要方法:
- 面部质量的组织病理学检查.
- 免疫组织化学分析包括光滑肌肉动蛋白和CD34.
- 对BRAF,NRAS和KIT突变进行分子测试.
主要成果:
- 诊断为高度上皮质性肌纤维素瘤 (FNCLCC 3 级).
- 瘤显示异质性,具有低等级的外围和高等级的中央组件.
- 瘤细胞对光滑肌肉动蛋白呈阳性,对其他标记物呈阴性;CD34突出显示了血管系统. 在测试的基因中没有发现突变.
结论:
- 皮肤上皮质神经纤维瘤是一种罕见的实体,需要仔细诊断.
- 结合组织病理学,免疫组织化学和分子分析的多模式方法至关重要.
- 早期诊断和管理对于有利的结果至关重要,这是9个月的随访没有复发或转移所证明的.
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