肝脏未分化胚胎性肉瘤临床病理特征分析并文献复习

肝脏 ›› 2025, Vol. 30 ›› Issue (1): 46-49.

肝脏未分化胚胎性肉瘤临床病理特征分析并文献复习

向娟, 曲延刚

445000 湖北恩施土家族苗族自治州中心医院

收稿日期:2023-11-22 出版日期:2025-01-31 发布日期:2025-03-10
通讯作者: 曲延刚,Email:1324322271@qq.com
基金资助:
恩施州科技计划青年人才项目(D20220068)

Clinico-pathological characteristics of undifferentiated embryonal sarcoma in the liver

XIANG Juan, QU Yan-Gang

Department of Pathology, the Central Hospital of Enshi Tujia and Miao Autonomous Prefecture, Hubei 445000, China

Received:2023-11-22 Online:2025-01-31 Published:2025-03-10
Contact: QU Yan-Gang, Email: 1324322271@qq.com

摘要/Abstract

摘要： 目的探讨肝脏未分化胚胎性肉瘤(UESL)的临床病理学特征、诊断、鉴别诊断及预后,并结合文献进行分析。方法采用回顾性分析,对一例肝脏未分化胚胎性肉瘤进行镜下组织学形态学观察、免疫组织化学的检测并随访,结合国内外文献分析其临床病理学特征及预后。结果患者男性,5岁,肝脏巨大肿物,大小15 cm×11 cm×9 cm。镜下肿瘤由轻-中度异型的梭形细胞或星状间叶性细胞组成,间质明显疏松伴黏液样变,胞质内外可见大小不等的嗜伊红色小体。可见多形性或瘤巨细胞。肿瘤细胞Vimentin、Bcl-2、CD99、CD10、CK7、Desmin呈阳性,嗜酸性小体PAS阳性,Ki67阳性指数60%。结论肝脏未分化胚胎性肉瘤是肝脏原始未分化间叶细胞构成的恶性肿瘤,属于高度恶性,最常发生于儿童。需要与间叶性错构瘤、胚胎性横纹肌肉瘤、肝母细胞瘤及胃肠道间质瘤鉴别。

关键词: 肝脏未分化胚胎性肉瘤, 免疫组化, 临床病理特征, 鉴别诊断

Abstract: Objective To investigate the clinicopathological characteristics, diagnosis, differential diagnosis and prognosis of undifferentiated embryonal sarcoma of the liver (UESL). Methods A retrospective analysis was collected and analyzed on a case of UESL, which focused on the following aspects: histomorphology, immunohistochemical staining and Clinicopathological manifestations. Meanwhile, the published literatures on UESL were reviewed. Methods The patient was a 5-year-old male child with a massive liver mass measuring 15 cm×11 cm×9 cm. Microscopically, the tumor cells were composed of light-moderate atypia spindle cells or stellate mesenchymal cells. Tissue in the interstitium was obviously loose with mucoid degeneration. Eosinophilic bodies were seen inside or outside the cytoplasm. Multinucleated or gigantiform cells could often be seen. Tumor cells were positive for Vimentin, Bcl-2, CD99, CD10, CK7, and Desmin. Eosinophilic bodies were positive for PAS. Ki67 positive index was 60%. Conclusion UESL is a highly malignant tumor composed of originally undifferentiated mesenchymal cells of the liver, most commonly occurred in children. It is necessary to differentiate UESL from hepatoblastoma, hepatic embryonal rhabdomyosarcoma, hepatic mesenchymal hamartoma, and gastrointestinal stromal tumor.

Key words: Undifferentiated embryonal sarcoma of the liver, Immunohistochemistry, Clinicopathological features, Differential diagnosis

向娟, 曲延刚. 肝脏未分化胚胎性肉瘤临床病理特征分析并文献复习[J]. 肝脏, 2025, 30(1): 46-49.

XIANG Juan, QU Yan-Gang. Clinico-pathological characteristics of undifferentiated embryonal sarcoma in the liver[J]. Chinese Hepatolgy, 2025, 30(1): 46-49.

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