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Primary Epithelial-myoepithelial Carcinoma of Thymus: First Case Report of the World and Literature Review
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摘要:
目的 探讨上皮肌上皮癌的临床症状、影像学特征、病理组织学特征和治疗方法。 方法 回顾性分析北京协和医院肿瘤内科收治的1例胸腺原发上皮肌上皮癌患者临床资料，并进行文献复习。 结果 患者为中年男性，是目前国内外报道的首例被诊断为胸腺原发上皮肌上皮癌的患者。该患者经手术、化疗、放疗、分子靶向治疗等综合治疗后仍出现疾病进展，从手术确诊至死亡时间为22个月。 结论 胸腺原发上皮肌上皮癌极为罕见，临床表现不典型，诊断主要依靠病理组织学检查，目前首选手术治疗，多学科综合治疗有助于延长患者的生存期。 Abstract:Objective To analyze the clinical, imaging and pathological features of patients with epithelial-myoepithelial carcinoma(EMC). Methods The data of a patient with primary EMC of the thymus in Peking Union Medical College Hospital was retrospectively analyzed, and the relevant literature was reviewed. Results The patient was a middle-aged male diagnosed with primary EMC of the thymus, and he was the first case currently reported at home and abroad. Even though the patient received comprehensive treatments including surgery, chemotherapy, radiotherapy and molecular targeted therapy, he still experienced disease progression, with a survival time of 22 months after surgery. Conclusions Primary EMC of the thymus is extremely rare and has atypical clinical manifestations. The diagnosis mainly depends on histopathological examination. Surgery is currently the major treatment and multidisciplinary treatment is very important to prolong the survival time of EMC patients. 作者贡献声明：王湘负责研究实施及论文撰写；邵亚娟、孙桂彬、周娜、甄宏楠、陈杰负责数据采集；赵林、白春梅负责研究指导、论文修订。利益冲突：所有作者均声明不存在利益冲突 -
图 2 患者术后病理图像
A.低倍镜下可见纤维分割的实性肿瘤细胞巢团，肿瘤细胞富含透明胞浆(HE，×100)；B.部分区域可见双层管状结构，内层为闰管样细胞，胞浆嗜酸性，外层呈单层或多层包绕，细胞体积较大，胞浆透明，核圆形(HE，×400)；C.CK19免疫组化染色呈胞膜着色；D.p63显示外层细胞核阳性
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