Primary myoepithelial carcinoma of palate.
Primary myoepithelial carcinoma of palate.
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原发性腭肌上皮癌
DOI：
10.1186/1477-7819-9-104
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发表时间：
2011-09-14
影响因子：
3.2
通讯作者：
Jiang S
中科院分区：
文献类型：
--
作者：
Ren J;Liu Z;Liu X;Li Y;Zhang X;Li Z;Yang Y;Yang Y;Chen Y;Jiang S
本研究旨在介绍一种罕见的肿瘤——起源于腭部的原发性肌上皮癌，并回顾其诊断标准、病理及临床特征、治疗方案和预后。
肌上皮瘤是主要或完全起源于肌上皮细胞的肿瘤。肌上皮瘤多发生于唾液腺，也可见于乳腺、皮肤和肺部。腭部肌上皮瘤的病例鲜有报道。肌上皮癌是肌上皮瘤的恶性对应肿瘤。腺肌上皮瘤也是一种与肌上皮瘤不同的疾病。免疫组织化学显示，肌上皮癌的肿瘤细胞不仅表达细胞角蛋白、上皮膜抗原（EMA）等上皮标志物，还表达钙调蛋白等平滑肌源性标志物。肌上皮分化的免疫组织化学标准是细胞角蛋白与一种或多种肌上皮免疫标志物（即S - 100蛋白、钙调蛋白、p63、胶质纤维酸性蛋白、maspin和肌动蛋白）双阳性。唾液腺和乳腺的肌上皮癌存在拷贝数增加和基因缺失。肌上皮癌的总体预后较差。良性肌上皮肿瘤很少复发或转移。首选治疗方案始终是切缘无瘤的完整切除，而局部放疗和化疗是可供参考的治疗选择。本文描述并讨论了一例罕见的腭部肌上皮癌病例的治疗及转归，并对肌上皮瘤的病理和临床特征进行了比较与探讨。
本病例报告有助于提高对肌上皮瘤的认识，提升其诊断和治疗水平。
The aim of this study was to present a rare neoplasm, Primary myoepithelial carcinoma arising from the palate, and to review its diagnostic criteria, pathologic and clinical characteristics, treatment options and prognosis. Myoepitheliomas are tumors arising from myoepithelial cells mainly or exclusively. Myoepitheliomas mostly occur in salivary glands, as well as in breast, skin, and lung. Case of myoepitheliomas in palate has rarely been reported. Myoepithelial carcinoma is malignant counterpart of myoepitheliomas. Adenomyoepithelioma is also a different disease from myoepitheliaomas. Immunohistochemically, tumor cells of myoepithelial carcinoma express not only epithelial markers such as cytokeratin, epithelial membrane antigen (EMA), but also markers of smooth muscle origin such as calponin. The immunohistochemical criteria of myoepithelial differentiation are double positive for both cytokeratins and one or more myoepithelial immunomarkers (i.e., S-100 protein, calponin, p63, GFAP, maspin, and actins). Myoepithelial carcinomas of salivary and breast demonstrate copy number gains and gene deletion. The overall prognosis of myoepithelial carcinoma is poor. There is rarely recurrence or metastasis in benign myoepithelial tumors. Complete excision with tumor-free margin is always the preferred treatment, while local radiation therapy and chemotherapy are suggestive treatment options. Here, a rare case of myoepithelial carcinoma arising from the palate has been described and discussed for the treatment and outcome. Pathological and clinical characters of myoepitheliomas are also compared and discussed. The case report serves to increase awareness and improve the index of diagnosis and treatment of myoepitheliomas.
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