首页> 外文期刊>Japanese Journal of Ophthalmology >Immunohistochemical diagnosis of a rare case of epithelioid malignant peripheral nerve sheath tumor with multiple metastases.
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Immunohistochemical diagnosis of a rare case of epithelioid malignant peripheral nerve sheath tumor with multiple metastases.

机译:免疫组织化学诊断罕见病例上皮样恶性周围神经鞘瘤多发转移。

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BACKGROUND: The epithelioid variant of malignant peripheral nerve sheath tumor (MPNST) is a rare tumor with poor prognosis that sometimes involves the head and neck. The diagnosis is based principally on the histological examination, and it is generally very difficult to reach the correct diagnosis. CASE: An 84-year-old Japanese woman presented with a tumor mass of 2 week's duration in the right medial canthal region. OBSERVATIONS: Although the tumor was excised surgically, metastases occurred three times on her face and head, and the patient died of distant systemic multiple metastases. In the histopathological analysis, the tumor showed a composite pattern comprising spindle or polygonal cells arranged in irregular bands, and a population of larger epithelioid cells in solid sheets and nests. In the immunohistochemical analysis, the tumor cells were positive for S-100 protein, vimentin, and nerve growth factor receptor (NGFR), and negative for cytokeratin and HMB 45 (melanoma-associated antigen). These findings confirmed the diagnosis of MPNST. CONCLUSIONS: Epithelioid MPNST has complex histopathological findings and histopathological features similar to other epithelioid tumors, especially malignant melanoma. Immunohistochemical examination using NGFR and HMB-45 is important in the differential diagnosis.
机译:背景:恶性周围神经鞘瘤(MPNST)的上皮样变体是一种罕见的预后不良的肿瘤,有时累及头部和颈部。诊断主要基于组织学检查,通常很难达到正确的诊断。病例:一位84岁的日本女性在右内侧can区域出现了持续2周的肿瘤块。观察结果:尽管通过外科手术切除了肿瘤,但她的面部和头部发生了3次转移,患者死于远处的全身多发转移。在组织病理学分析中,肿瘤显示出复合模式,包括梭形或多边形细胞排列成不规则带,以及大量的上皮样细胞在实心的表层和巢中。在免疫组织化学分析中,肿瘤细胞的S-100蛋白,波形蛋白和神经生长因子受体(NGFR)呈阳性,而细胞角蛋白和HMB 45(黑色素瘤相关抗原)呈阴性。这些发现证实了MPNST的诊断。结论:上皮样MPNST具有与其他上皮样肿瘤特别是恶性黑色素瘤相似的复杂的组织病理学发现和组织病理学特征。使用NGFR和HMB-45进行的免疫组织化学检查在鉴别诊断中很重要。

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