Guo Hui, Chen Shuaiqi, Liu Shukun, Wang Kaixuan, Liu Erpeng, Li Faping, Hou Yuchuan
Department of Urology, First Hospital of Jilin University, Changchun, Jilin, 130021, China.
Department of Urology, The First Affiliated Hospital of Xinxiang Medical University, Xinxiang, Henan, 453100, China.
BMC Urol. 2017 Apr 4;17(1):24. doi: 10.1186/s12894-017-0217-3.
Members of the Ewing's sarcoma family of tumor (ESFT) are malignant neoplasms and rarely observed in the adrenal gland.
We report an extremely exceptional case of ESFT rising from the adrenal gland in a 57-year-old Chinese man. The patient was hospitalized with abdominal swelling for 2 months. Computed tomography (CT) scan revealed a nearly-circular mass measuring about 8.1 × 10.6 cm in the right adrenal region. The patient underwent right adrenal resection. Histopathologic examination found the tumor was composed of small round blue cells forming typical Homer-Wright rosettes in focal area. The immunohistochemical analysis confirmed the case to be ESFT, which was positive for membranous CD99 and nuclear FLI-1. The patient was scheduled for four courses of large doses of chemotherapy and died for cancer metastasis one year later after surgery.
Histopathological evidence of Homer-Wright rosettes and immunohistochemical markers positivity, such as CD99 and FLI-1, are valuable factors for ESFT diagnosis, although cytogenetic analysis is considered as the gold standard. Complete surgery is the treatment of choice for ESFT and adjuvant radiotherapy and combination chemotherapy can significantly improve the survival rate of postoperative patients.
尤因肉瘤家族肿瘤（ESFT）成员为恶性肿瘤，很少见于肾上腺。
我们报告了一例极为罕见的ESFT病例，发生于一名57岁中国男性的肾上腺。患者因腹部肿胀住院2个月。计算机断层扫描（CT）显示右肾上腺区有一个近圆形肿块，大小约为8.1×10.6厘米。患者接受了右肾上腺切除术。组织病理学检查发现肿瘤由小圆形蓝色细胞组成，在局部区域形成典型的霍纳-赖特玫瑰花结。免疫组织化学分析证实该病例为ESFT，膜性CD99和核FLI-1呈阳性。患者计划接受四个疗程的大剂量化疗，术后一年因癌症转移死亡。
霍纳-赖特玫瑰花结的组织病理学证据以及CD99和FLI-1等免疫组织化学标志物阳性是ESFT诊断的重要因素，尽管细胞遗传学分析被视为金标准。完整手术是ESFT的首选治疗方法，辅助放疗和联合化疗可显著提高术后患者的生存率。
