肝脏未分化胚胎性肉瘤临床病理学特征分析及文献复习

摘要/Abstract

摘要： 探讨肝脏未分化胚胎性肉瘤（UESL）的临床病理学特征、诊断、鉴别诊断及相关分子遗传学特征。回顾性收集并分析2020—2022年河南省肿瘤医院确诊的UESL 3例,复查相关临床、影像及病理资料,采用免疫组织化学法检测肿瘤免疫表型。随访患者并结合相关文献分析其相关分子遗传学特征。本组3例中男性2例,女性1例,平均年龄9.3岁（6~13岁）。镜下黏液样基质中可见卵圆形或梭形细胞呈团片状或束状排列,散在大的多形性或瘤巨细胞;细胞密度不等,部分区域细胞稀疏,部分区域细胞密集;细胞质内或细胞质外可见特征性嗜酸性小体,核分裂象易见;间质血管丰富,出血坏死明显。免疫组织化学检测3例Vimentin均呈阳性,CK、CD68呈局灶阳性,PAS阳性,Ki-67增殖指数为40%~90%阳性;2例P53全阴性显示无义突变表达,1例阳性（80%）显示错义突变表达。3例均行手术切除及术后化疗,其中1例辅助放射治疗。目前随访时间22~57个月,其中2例分别在随访15、24个月时复发。检索国内外有关UESL的文献报道并总结发现目前关于UESL的发病机制尚未明确,文献中报道较多的为19q13.4的染色体重排,包括t（11;19）（q11;q13.3/13.4）易位和（19）（q13.4）的增加。也有少数病例报道UESL存在TP53基因的点突变。UESL罕见,易发生于儿童,预后有待于长期随访及大数据统计。临床症状及影像学不具有特异性,确诊需依靠病理学。治疗主要为手术和化疗,相关分子检测及靶向治疗有待于更多临床经验的积累。

关键词: 未分化胚胎性肉瘤, 肝脏, 病理学, 临床特性

张银苹, 王远, 王媛媛, 张冰, 高歌, 夏庆欣. 肝脏未分化胚胎性肉瘤临床病理学特征分析及文献复习[J]. 肝癌电子杂志, 2025, 12(1): 15-18.

Zhang Yinping, Wang Yuan, Wang Yuanyuan, Zhang Bing, Gao Ge, Xia Qingxin. Clinicopathological features and literature review of undifferentiated embryonal sarcoma of the liver[J]. Electronic Journal of Liver Tumor, 2025, 12(1): 15-18.

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