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唾液腺的硬化性粘膜皮质癌

Bacem Khalele Othman¹, Martina Bradová^1,2, Roderick H W Simpson³

¹Department of Pathology, Faculty of Medicine in Pilsen, Charles University, Pilsen, Czech Republic.

Virchows Archiv : an international journal of pathology

|November 13, 2024

概括

硬化性粘膜腺癌 (SMEC) 是一种罕见的唾液腺癌. 分子分析证实,SMEC是一种低度粘膜皮细胞癌的变体,具有良好的结果.

科学领域:

病理学病理学病理学
在瘤学瘤学.
分子生物学分子生物学

背景情况:

硬化性粘膜皮质癌 (SMEC) 是一种罕见的,低度的唾液腺恶性瘤.
SMEC的特点是显著的纤维化和炎症透,通常与稀缺的细胞缺陷.
鉴于其独特的组织学特征,区分SMEC与其他唾液腺瘤可能具有挑战性.

研究的目的:

确定唾液腺SMEC的诊断标准.
研究SMEC的分子特征,特别是MAML2基因重组.
评估唾液腺SMEC患者的临床结果.

主要方法:

对25例未发表的唾液腺SMEC病例进行了回顾性分析.
组织病理学审查侧重于诊断挑战,如原性流体和不显眼的粘膜细胞.
分子分析包括光在位杂交 (FISH) 和下一代测序 (NGS) 用于MAML2基因重组.

主要成果:

在可分析的唾液SMEC病例中,86% (18/21) 发现了MAML2基因重组.
两个病例 (8%) 呈现出显著的氨酸,三个病例 (12%) 显示出丰富的IgG4阳性血细胞.
随访数据表明,唾液SMEC患者的结果有利.
唾液SMECs在遗传上与甲状腺SMECs不同.

结论:

唾液腺SMEC是一种罕见的常规低度粘膜皮瘤癌的罕见变体.
MAML2基因重组是唾液SMEC中常见的分子特征.
唾液SMEC表现出有利的临床行为.
唾液腺和甲状腺的SMEC具有不同的遗传特征.

关键词:

进行MAML2重组.在IgG4的基础上,IgG4 类似形体的 stromal 纤维化.美国中小企业和中小企业 (SMEC)唾液腺唾液腺是什么硬化性粘膜皮性癌瘤的癌症.硬化症硬化症硬化症组织埃索诺菲利亚 (Tissue Eosinophilia) 是一种

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