肝原发性多形性未分化肉瘤3例临床病理特点分析及文献复习

肝癌电子杂志 ›› 2025, Vol. 12 ›› Issue (1): 10-14.

肝原发性多形性未分化肉瘤3例临床病理特点分析及文献复习

李卓, 宫丽华, 马沛卿, 王冰凝, 李金, 鲁海珍^*

国家癌症中心/国家肿瘤临床医学研究中心/中国医学科学院北京协和医学院肿瘤医院病理科,北京 100021

收稿日期:2024-02-21 出版日期:2025-03-31 发布日期:2025-05-08
通讯作者: ^*鲁海珍,E-mail: lizhuo@cicams.ac.cn
基金资助:
中国医学科学院医学与健康科技创新工程项目临床与转化研究医学专项（2024-I2M-C&T-B-069）

Clinicopathological characteristics of three cases of liver primary pleomorphic undifferentiated sarcoma and literature review

Li Zhuo, Gong Lihua, Ma Peiqing, Wang Bingning, Li Jin, Lu Haizhen

Received:2024-02-21 Online:2025-03-31 Published:2025-05-08

摘要/Abstract

摘要： 探讨肝原发性多形性未分化肉瘤（HUPS）的临床病理特点、诊断与鉴别诊断与分子病理学改变。选取2017年3月至2024年12月中国医学科学院肿瘤医院手术切除且经外科根治性术后明确病理诊断的3例HUPS患者,分析其临床病理学特点。3例患者中1例男性,2例女性,发病年龄54~65岁。病理学显微镜镜下肿瘤细胞形态多样,均含有多核瘤巨细胞,异型性显著;免疫组织化学染色结果呈Vim阳性,Ki-67高增殖活性（50%、60%、65%）,肌源性等提示明确分化方向的标志物SMA和S-100等均呈阴性结果。二代测序结果部分病例呈TP53基因突变,3例无重叠性的基因突变及融合改变检出。HUPS是一种极为罕见的软组织恶性肿瘤,形态学上难以与肉瘤样癌及多种高级别肉瘤鉴别,其病理诊断需结合免疫组织化学及分子病理学改变综合判断。

李卓, 宫丽华, 马沛卿, 王冰凝, 李金, 鲁海珍. 肝原发性多形性未分化肉瘤3例临床病理特点分析及文献复习[J]. 肝癌电子杂志, 2025, 12(1): 10-14.

Li Zhuo, Gong Lihua, Ma Peiqing, Wang Bingning, Li Jin, Lu Haizhen. Clinicopathological characteristics of three cases of liver primary pleomorphic undifferentiated sarcoma and literature review[J]. Electronic Journal of Liver Tumor, 2025, 12(1): 10-14.

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