Abstract
患者女，51岁。有高血压病史，劳累时感腰部酸痛，休息后缓解。体检时B超和腹部增强CT检查发现左肾上腺区占位，行腹腔镜左肾上腺肿瘤切除术，术后病理检查：镜下肿瘤细胞呈梭形，异型性明显，呈疏密交替的束状排列，局部可见血管外皮瘤样结构和地图样坏死；免疫组织化学结果显示：肿瘤细胞H3K27me3表达缺失，S-100蛋白个别细胞阳性，SOX10阴性，横纹肌标志物结蛋白、Myogenin和MyoD1弥漫阳性。荧光原位杂交检测结果显示MDM2基因扩增阴性。本例诊断为肾上腺恶性外周神经鞘膜瘤伴完全性异源性横纹肌母细胞分化（恶性蝾螈瘤），按照法国癌症中心联盟（FNCLCC）分级系统归为3级。患者术后未行放化疗，随访7个月，情况尚良好，复查未见复发转移证据，目前密切随访中。.
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