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肝脏上皮样血管肉瘤:两例报告并文献复习

Epithelioid angiosarcoma of the liver: report of two cases and review of the literature.

作者信息

Long Manmei, Shali Wumairehan, Huang Qiongyi, Bao Yongyang, Chen Ying, Rao Yaming, Zhou Henghua, Liu Qiang, He Qiao, Zheng Chuyi, Liu Wenyong, Zhu Yanbo

机构信息

Department of Pathology, Shanghai Ninth People's Hospital, School of Medicine, Shanghai Jiao Tong University, Center for Specialty Strategy Research of Shanghai Jiao Tong University China Hospital Development Institute Shanghai, China.

Department of General Surgery, Shanghai Ninth People's Hospital, School of Medicine, Shanghai Jiao Tong University, Center for Specialty Strategy Research of Shanghai Jiao Tong University China Hospital Development Institute Shanghai, China.

出版信息

Int J Clin Exp Pathol. 2018 Jun 1;11(6):3191-3198. eCollection 2018.

Abstract

Angiosarcoma is a malignant tumor of endothelial origin. Epithelioid angiosarcoma is a subtype of angiosarcoma, in which the malignant endothelial cells have a predominantly epithelioid appearance. So far, few cases of primary hepatic epithelioid andiosarcoma (PHEA) have been described. In this case report, we describe two rare cases of PHEA. Microscopically, the tumors were consistently composed of atypical epithelioid cells with vesicular nuclei, prominent nucleoli, and eosinophilic cytoplasm. One patient had metastatic disease and underwent palliative hepatic surgery following radiotherapy and chemotherapy, and had a postoperative survival time of 12 months, while the other patient is still alive after tumor resection. PHEA is an aggressive malignant tumor with a high rate of metastasis.

摘要

血管肉瘤是一种起源于内皮细胞的恶性肿瘤。上皮样血管肉瘤是血管肉瘤的一种亚型,其中恶性内皮细胞主要呈上皮样外观。到目前为止,原发性肝上皮样血管肉瘤(PHEA)的病例报道很少。在本病例报告中,我们描述了两例罕见的PHEA病例。显微镜下,肿瘤始终由具有泡状核、明显核仁及嗜酸性细胞质的非典型上皮样细胞组成。一名患者发生了转移,在放疗和化疗后接受了姑息性肝脏手术,术后存活时间为12个月,而另一名患者在肿瘤切除后仍然存活。PHEA是一种侵袭性恶性肿瘤,转移率很高。

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