基于临床实践的6种子宫罕见间叶性肿瘤临床病理学特征分析  

Analysis on clinicopathological features of 6 types of rare uterine mesenchymal tumors based on clinical practice

作　　者：谢乐[1] 曾敏[1] 李红玲 徐园园 黄颖欣 韦旭旋 张锦晖 毛荣军[1] XIE Le;ZENG Min;LI Hongling;XU Yuanyuan;HUANG Yingxin;WEI Xuxuan;ZHANG Jinhui;MAO Rongjun(Department of Pathology,Foshan Hospital of Traditional Chinese Medicine,Guangdong 528000,China)

机构地区：[1]佛山市中医院病理科,广东528000

出　　处：《中国临床新医学》2024年第7期733-739,共7页CHINESE JOURNAL OF NEW CLINICAL MEDICINE

基　　金：佛山市“十四五”医学重点专科项目(编号:FSZD145010);2022年佛山市自筹经费类科技创新项目入库培育项目(医学类科技攻关)(编号:2220001005384)。

摘　　要：目的探讨子宫罕见间叶性肿瘤的临床病理学特征。方法收集2020年1月至2024年5月佛山市中医院确诊的子宫疑难罕见间叶性肿瘤病例资料,根据佛山市中医院病理科疑难罕见病变的拟定标准,共收集到6种子宫罕见间叶性肿瘤,并结合相关文献进行总结和分析。结果该文对6种病变[包括平滑肌瘤合并脂肪母细胞瘤,多形性腺瘤基因1(PLAG1)重排纤维黏液样肉瘤,胚胎型神经外胚层肿瘤/中枢型原始神经外胚层肿瘤,非特指DICER1突变相关肉瘤,复合性子宫高级别肉瘤伴胚胎性横纹肌肉瘤、纤维肉瘤及神经外胚层样分化,MXI1-NUTM1融合肉瘤等]进行临床、形态学、免疫组化及分子检测结果特征描述。结论子宫间叶性肿瘤相对少见,但多种子宫外间叶性肿瘤仍可发生于子宫,而且临床病理亚专科分级诊断存在一些不足,导致存在误诊漏诊的潜在风险,精准诊断有赖于临床、形态学、免疫组化及分子检测的综合评估。Objective To explore the clinicopathological features of rare uterine mesenchymal tumors.Methods The case data of rare and intractable uterine mesenchymal tumors definitely diagnosed in Foshan Hospital of Traditional Chinese Medicine from January 2020 to May 2024 were collected.According to the proposed criteria of rare and intractable pathological changes in the Department of Pathology,Foshan Hospital of Traditional Chinese Medicine,6 types of rare uterine mesenchymal tumors were collected,and the relevant literature was summarized and analyzed.Results In this study,six lesions[including leiomyoma combined with lipoblastoma,pleomorphic adenoma gene 1(PLAG1)rearranged fibromyxoid sarcoma,embryonal neuroectodermal tumor/central type primitive neuroectodermal tumor,unspecifiedDICER1mutation-related sarcoma,composite uterine high-grade sarcoma with embryonal rhabdomyosarcoma and fibrosarcoma and neuroectodermal differentiation,andMXI1-NUTM1fusion sarcoma]were selected and the features of their clinical,morphological,immunohistochemical and molecular detection results were described.Conclusion Uterine mesenchymal tumors are relatively rare,but various types of mesenchymal tumors originating outside the uterus still occur in the uterus,and due to some limitations in sub-specialty grading diagnosis of clinical pathology,there is a potential risk of misdiagnosis and missed diagnosis,and accurate diagnosis of these mesenchymal tumors relies on the comprehensive evaluation of their clinical,morphological,immunohistochemical and molecular detections.

关 键 词：子宫肿瘤 间叶性肿瘤 分子遗传学 疑难罕见病变 

分 类 号：R730.2[医药卫生—肿瘤]