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子宫炎性肌纤维母细胞肿瘤32例临床病理及分子遗传学特征分析 被引量:1

Uterine inflammatory myofibroblastic tumor:clinicopathologic, and molecular analysis of 32 cases
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摘要 目的探讨子宫炎性肌纤维母细胞肿瘤(uterine inflammatory myofibroblastic tumor,UIMT)的临床病理学及分子学特征。方法回顾性分析32例UIMT的临床病理资料,行HE染色、免疫组化染色及分子检测,并复习相关文献。结果32例患者年龄35~71岁(平均45.69岁;中位年龄45岁),肿瘤最大径1.8~15.5 cm(平均8.36 cm;中位7 cm)。镜检:均为梭形细胞肿瘤,细胞密度不等,细胞核长梭形,大部分类似于平滑肌细胞核,胞质嗜酸性,核分裂象1~4个/10 HPF;多数轻度异型,4例局部细胞中度异型;1例见坏死,细胞中度-重度异型,细胞呈上皮样,可见核分裂象,符合上皮样炎性肌纤维母细胞肉瘤。免疫表型:瘤细胞SMA、H-Caldesmon、Calponin、desmin、ALKp80、ALK(D5F3)及p16均阳性,Ki-67增殖指数为1%~15%。FISH检测示32例均见ALK基因分离信号;2例外院会诊病例行NGS检测,发现ALK-TIMP3及ALK-TPM3融合基因。结论UIMT属于中间型软组织肿瘤,确诊需结合临床病理特征、免疫表型及ALK基因检测。治疗方法有肿物切除、全子宫切除等,预后较好。 Purpose To investigate the clinical and molecular pathological characteristics of uterine inflammatory myofibroblastic tumor(UIMT).Methods The clinicopathological data of 32 UMIT cases,who underwent HE staining,immunohistochemical staining,and molecular testing,were retrospectively reviewed,and relevant literatures were reviewed.Results There were 32 females,aged 35-71 years(mean 45.69 years;median 45 years).The diameter of masses ranged from 1.8 cm-15.5 cm(average 8.36 cm,median 7 cm).Microscopically,all cases were spindle cell tumors with varying densities.The tumor cells were spindle shaped nuclei,mostly resembling smooth muscle nuclei.The cytoplasm was eosinophilic,and the mitosis were 1-4/10HPF.Most cases were mild atypia,and four were moderate.Focally necrosis was observed in one case.The tumor cells were moderate to severe atypia,with epithelioid features.The mitosis were observed,and consistent with epithelioid inflammatory myofibroblastoma sarcoma.Immunophenotype,tumor cells showed positive for SMA,H-Caldesmon,Calponin,desmin,ALKp80,ALK(D5F3),and p16.Ki-67 proliferation index were 1%to 15%.ALK gene apart signal was detected in all cases.Two case were performed with NGS for ALK-TIMP3,including ALK-TPM3 fusion genes were detected.Conclusion UIMT is an intermediate soft tissue tumor.The diagnosis should be combined with clinical pathology,immunophenotype and ALK gene detection.The treatment includes surgically resection,myomectomy(uterus retained),hysterectomy,etc.The prognosis is good.
作者 黄海建 陈小岩 郑松龄 钟定荣 HUANG Haijian;CHEN Xiaoyan;ZHENG Songling;ZHONG Dingrong(Department of Pathology,Fujian Provincal Hospital/Provincial Clinical Medical College of Fujian Medical University Fuzhou 350001,China;Department of Pathology,Taijiang Hospital,Fuzhou 350009,China;Department of Pathology,China-Japan Friendship Hospital,Beijing 100029,China)
出处 《临床与实验病理学杂志》 CAS 北大核心 2023年第10期1207-1211,共5页 Chinese Journal of Clinical and Experimental Pathology
关键词 子宫肿瘤 炎性肌纤维母细胞肿瘤 免疫组织化学 基因检测 uterine neoplasms inflammatory myofibroblastic tumor UIMT immunohistochemistry gene detection
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