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肝胚胎性横纹肌肉瘤合并肝细胞癌的临床病理分析

Clinicopathological analysis of concomitant hepatic embryonal rhabdomyosarcoma and hepatocellular carcinoma.

作者信息

Li Haifeng, Zhang Yanyang, Pan Yuhang, Hui Dayang, Chen Jianning, Jin Yi

机构信息

Department of Pathology, The Third Affiliated Hospital of Sun Yat-Sen University, Guangzhou 510630, China; Guangdong Key Laboratory of Liver Disease Research, The Third Affiliated Hospital of Sun Yat-Sen University, Guangzhou 510630, China.

Department of Interventional Radiology, The Third Affiliated Hospital of Sun Yat-Sen University, Guangzhou 510630, China; Guangdong Key Laboratory of Liver Disease Research, The Third Affiliated Hospital of Sun Yat-Sen University, Guangzhou 510630, China.

出版信息

Pathol Res Pract. 2017 Aug;213(8):1014-1018. doi: 10.1016/j.prp.2017.04.015. Epub 2017 Apr 20.

Abstract

Hepatic embryonal rhabdomyosarcoma (ERMS) is extremely rare. Here we report the simultaneous occurrence of hepatocellular carcinoma (HCC) and ERMS of the liver in a 40-year-old man without any symptoms. Macroscopically, the mass was composed of two different tumors. The large tumor was 4.5×4×4cm and was poorly circumscribed and soft in the central region of left lateral lobe of the liver with apparently focal necrosis. The small tumor, with diameter of 1cm, was adjacent to the large tumor without clear boundary. Histologically, the large tumor was composed of numerous spindle-shaped or round cells with brightly eosinophilic cytoplasm as well as pathologic mitosis. Immunohistochemical staining was positive for MyoD1 and myogenin in nuclear testing. However, in the small tumor, cells demonstrated hepatocyte differentiation and were focally positive for HepPar1. A diagnosis of concomitant ERMS and HCC of the liver was made. The patient received no adjuvant treatment after hepatic left lateral lobectomy. The regular follow-up observation conducted by imaging examinations displayed that there was no sign of recurrence or metastasis of the mass over 32 months. To our knowledge, this is the first case report of ERMS of the liver associated with HCC. The diagnosis can only be made by pathological examination. The primary therapy method for this tumor is operative resection.

摘要

肝胚胎性横纹肌肉瘤(ERMS)极为罕见。在此,我们报告一名40岁无症状男性同时发生肝脏肝细胞癌(HCC)和ERMS。大体上,肿块由两种不同肿瘤组成。较大肿瘤大小为4.5×4×4cm,边界不清,位于肝左外叶中央区域,质地柔软,有明显局灶性坏死。较小肿瘤直径1cm,与较大肿瘤相邻,边界不清。组织学上,较大肿瘤由众多梭形或圆形细胞组成,胞质嗜酸性明显,可见病理性核分裂。免疫组化染色显示核检测中MyoD1和肌生成素呈阳性。然而,在较小肿瘤中,细胞显示肝细胞分化,HepPar1呈局灶性阳性。诊断为肝脏同时发生ERMS和HCC。患者在肝左外叶切除术后未接受辅助治疗。通过影像学检查进行的定期随访观察显示,32个月来肿块无复发或转移迹象。据我们所知,这是首例肝脏ERMS合并HCC的病例报告。该诊断只能通过病理检查做出。该肿瘤的主要治疗方法是手术切除。

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