文献类型：期刊文献

中文题名：非典型和恶性血管球瘤3例临床病理分析

英文题名：Atypical and malignant glomus tumors:a clinicopathological analysis of three cases

作者：何倩倩[1,2];陈哲宇[1,3];邵彩玲[1,2];张鹏飞[1,2];杨艳丽[1]

第一作者：何倩倩

机构：[1]解放军联勤保障部队第九四〇医院病理科,兰州730050;[2]甘肃中医药大学第一临床医学院,兰州730000;[3]西北民族大学,兰州730030

第一机构：解放军联勤保障部队第九四〇医院病理科,兰州730050

年份：2026

卷号：33

期号：3

起止页码：344

中文期刊名：诊断病理学杂志

外文期刊名：Chinese Journal of Diagnostic Pathology

基金：甘肃省卫生健康行业科研项目(GSWSKY2024-59);甘肃省科技计划项目(20JR10RA003)。

语种：中文

中文关键词：血管球瘤病;恶性潜能未定的血管球瘤;恶性血管球瘤;临床病理特征;鉴别诊断

外文关键词：glomangiomatosis;GT-UMP;MGT;clinicopathological features;differential diagnosis

摘要：目的探讨非典型和恶性血管球瘤的临床病理学、鉴别诊断及治疗策略,以提高病理医师对本病的认识。方法收集联勤保障部队第九四〇医院病理科确诊的3例非典型和恶性血管球瘤,分别为血管球瘤病(glomangiomatosis)、恶性潜能未定的血管球瘤(GT-UMP)和恶性血管球瘤(MGT),回顾性总结患者的临床病理资料,进行形态学观察、免疫组织化学染色及分子学检测,并进行相关文献复习。结果3例均为女性,发病年龄50~62岁,3例分别发生于胸膜、胃体和小腿。免疫组化均表达SMA和Syn,Ki-67增殖指数为10%~35%;分子改变:胸膜和小腿部位病变行二代基因测序(NGS)检测,检出NOTCH2基因间区融合。结论血管球瘤病、GT-UMP表现为潜在和低度恶性生物学行为,恶性血管球瘤是一种高度侵袭性肿瘤,肿块的完全切除是最重要的预后因素,均可复发,其中MGT的转移率较高,因此应正确诊断、及时治疗及定期随访。
Objective To investigate the clinicopathological features,differential diagnosis,and treatment strategies of three cases of atypical and malignant glomus tumors,including glomangiomatosis,glomus tumor of uncertain malignant potential(GT-UMP),and malignant glomus tumor(MGT),in order to enhance pathologists'understanding of these entities.Methods Three cases of atypical and malignant glomus tumors diagnosed in the 940th Hospital of PLA Joint Logistic Support Force were collected.The clinicopathological data of the patients were retrospectively summarized,and morphological observation,immunohistochemical staining,and molecular testing were performed,along with a review of relevant literature.Results All three patients were female,aged 50-62 years.The tumors occurred in the pleura,gastric body,and lower leg,respectively.Immunohistochemical analysis showed positive expression of SMA and Syn in all cases,with a Ki-67 proliferation index ranging from 10%to 35%.Molecular testing using next-generation sequencing(NGS)for the pleural and lower leg lesions revealed NOTCH2 intergenic fusions.Conclusion Glomangiomatosis and GT-UMP exhibit potential or low-grade malignant biological behavior,while MGT is a highly aggressive tumor.Complete resection of the mass is the most critical prognostic factor.All subtypes can recur,with MGT having a higher metastasis rate.Therefore,accurate diagnosis,timely treatment,and regular follow-up are essential.