S100-negative epithelioid malignant peripheral nerve sheath tumor with possible perineurial differentiation
S100-negative epithelioid malignant peripheral nerve sheath tumor with possible perineurial differentiation
复制标题
S100阴性上皮样恶性周围神经鞘瘤，可能伴有神经周围分化
DOI：
10.1007/s00428-021-03218-y
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发表时间：
2021
期刊：
影响因子：
3.5
通讯作者：
Takeuchi K
中科院分区：
文献类型：
--
作者：
Yamashita K;Funauchi Y;Hayakawa K;Ae K;Matsumoto S;Ikuta K;Nishida Y;Ueno T;Shimoyama Y;Hiruta N;Machinami R;Kawachi H;Takeuchi K
上皮样恶性外周神经鞘膜瘤（MPNST）是一种罕见的MPNST亚型，由上皮样细胞和丰富的胞浆组成。目前，S100蛋白和SOX 10的强和弥漫性免疫染色通常被认为是上皮样MPNST的特征性特征。然而，上皮样形态的恶性肿瘤，从周围神经或预先存在的良性神经鞘肿瘤应被视为上皮样MPNST时，他们没有表现出明确的特征，导致其他具体的诊断。在这里，我们描述了3例上皮样MPNST在周围神经或神经鞘瘤是阴性的S100蛋白和SOX 10表达。相反，这些肿瘤在不同程度上对EMA、GLUT 1、claudin 1和细胞角蛋白呈阳性，而通过免疫组织化学，所有这些肿瘤均保留SMARCB 1和H3 K27 me 3。EMA、GLUT 1和claudin 1是神经束膜细胞分化的已知标志物;因此，它们可能代表具有神经束膜细胞分化的上皮样MPNST。
Epithelioid malignant peripheral nerve sheath tumor (MPNST) is a rare subtype of MPNST composed of epithelioid cells with abundant cytoplasm. Currently, strong and diffuse immunostaining for S100 protein and SOX10 is generally regarded as a characteristic feature of epithelioid MPNST. However, malignant tumors with epithelioid morphology that arise from a peripheral nerve or a pre-existing benign nerve sheath tumor should be regarded as epithelioid MPNSTs when they do not show characteristic features that definitively lead to other specific diagnoses. Here, we describe 3 cases of epithelioid MPNST in the peripheral nerve or schwannoma that was negative for S100 protein and SOX10 expression. Instead, these tumors were positive for EMA, GLUT1, claudin 1, and cytokeratin to varying degrees, while all of them retained SMARCB1 and H3K27me3 by immunohistochemistry. EMA, GLUT1, and claudin 1 are known markers of perineurial cell differentiation; thus, they could possibly represent epithelioid MPNST with perineurial cell differentiation.