“单纯型”黏液表皮样癌的挑战——纯梭形-透明细胞形态罕见病例报告。
The Challenge of "Monomorphic" Mucoepidermoid Carcinoma-Report of a Rare Case with Pure Spindle-Clear Cell Morphology.
机构信息
Department of Pathology, National University Health System, 5 Lower Kent Ridge Road, 119074, Singapore, Singapore.
Department of Pathology, Singapore General Hospital, Singapore, Singapore.
出版信息
Head Neck Pathol. 2023 Sep;17(3):864-870. doi: 10.1007/s12105-023-01547-9. Epub 2023 Apr 4.
BACKGROUND
Mucoepidermoid carcinoma is a malignant salivary gland tumor which, in most cases, is composed of variable proportions of mucous, epidermoid, and intermediate cells.
METHODS
We report a case of parapharyngeal mucoepidermoid carcinoma with highly unusual ("monomorphic") light microscopic features as well as atypical immunohistochemical properties. Molecular analysis was performed using the TruSight RNA fusion panel.
RESULTS
The tumor featured heretofore undescribed histopathological features: sheets and nests composed of monomorphic neoplastic (plump spindle to epithelioid) cells with no mucous, intermediate, glandular/columnar, or any other cell type identified. The neoplastic cells displayed variable clear cell change and only expressed cytokeratin 7. Despite this non-classical morphology, the presence of the classical CRTC1::MAML2 fusion was demonstrated.
CONCLUSIONS
Mucoepidermoid carcinoma featuring a uniform ("monomorphic") population of neoplastic cells is a novel observation. A confident diagnosis of mucoepidermoid carcinoma can be made upon detection of the CRTC1/3::MAML2 fusion. Our case increases the spectrum of histopathological appearances that mucoepidermoid carcinoma may display.
背景
黏液表皮样癌是一种恶性涎腺肿瘤，在大多数情况下，由不同比例的黏液、表皮样和中间细胞组成。
方法
我们报告了一例咽旁黏液表皮样癌，其具有非常罕见的（“同形性”）光镜特征以及非典型的免疫组织化学特征。使用 TruSight RNA 融合 panel 进行了分子分析。
结果
肿瘤具有前所未有的组织病理学特征：由同形性肿瘤细胞（肥胖的纺锤形至上皮样）组成的片状和巢状结构，没有黏液、中间、腺/柱状或任何其他细胞类型。肿瘤细胞显示出不同程度的透明细胞改变，仅表达细胞角蛋白 7。尽管存在这种非典型形态，但检测到了经典的 CRTC1::MAML2 融合。
结论
以均匀（“同形性”）的肿瘤细胞群为特征的黏液表皮样癌是一种新的观察结果。在检测到 CRTC1/3::MAML2 融合时，可以做出明确的黏液表皮样癌诊断。我们的病例增加了黏液表皮样癌可能表现出的组织病理学表现谱。
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