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子宫炎性肌纤维母细胞肿瘤临床及分子病理学特征

Clinical and molecular pathological features of uterine inflammatory myofibroblastic tumor

摘要:

目的:探讨子宫炎性肌纤维母细胞肿瘤(UIMT)的临床病理及分子病理学特点。方法:收集同济大学附属第一妇婴保健院2019—2020年6例UIMT患者的临床及病理资料,分析其临床及分子病理学特点。结果:6例患者年龄14~65岁;肉眼及临床检查肿瘤位于子宫肌壁间、黏膜下,直径2.5~6.0 cm,4例肿瘤切面略灰黄、灰白,2例肿瘤切面灰白、灰褐相间,质地较硬;镜下主要有两种形态:纤维型和黏液型,纤维型呈纤维瘤病样,主要表现为富于细胞的束状纤维母细胞或肌纤维母细胞增生,细胞核呈胖梭形伴不同程度异型性;黏液型呈筋膜炎样,主要表现为黏液样或水肿性基质中见胖梭形肿瘤细胞。在肿瘤间质中可见淋巴细胞和浆细胞浸润,肿瘤细胞异型性轻至中度,核分裂象(2~4)个/10 HPF。免疫组织化学5例显示肿瘤细胞间变性淋巴瘤激酶(ALK)阳性,1例显示ALK阴性;荧光原位杂交(FISH)检测证实5例肿瘤细胞存在ALK基因重排,1例肿瘤细胞ALK基因重排阴性。结论:UIMT是一类中间型肿瘤,具有局部复发和转移的风险。镜下瘤细胞呈梭形细胞形态,易于和子宫平滑肌肿瘤、子宫内膜间质肿瘤混淆。免疫组织化学ALK阳性或FISH检测ALK基因重排有助于明确诊断。

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abstracts:

Objective:To evaluate the clinical and molecular pathologic features of uterine inflammatory myofibroblastic tumor (UIMT).Methods:Six UIMT cases collected at Department of Pathology, Shanghai First Maternity and Infant Hospital, School of Medicine, Tongji University from 2019 to 2020. They were analyzed for their general characteristics and clinicopathologic features. ALK rearrangements were detected by fluorescence in situ hybridization.Results:The age of the six patients ranged from 14 to 65 years, the tumors ranged in size from 2.5 to 6.0 cm. The masses were intramural or submucosal in location. Most of them (4/6) were white with yellow foci, and two (2/6) were white with tan foci. Other features noted included a soft or firm appearance. The fasciitis-like pattern of UIMT had myxoid stroma around the spindle cells and inflammatory cells. The ganglion-like pattern showed either fascicular or storiform architecture with diffuse growth. Nuclear atypia was mild or moderate. Mitoses ranged from 2 to 4 per 10 high-power fields. Five tumors were ALK-positive with granular cytoplasmic staining by immunohistochemistry. ALK rearrangements were detected in five cases but was absent in one case.Conclusions:UIMT is an intermediate grade soft tissue tumor, a minority may present with extrauterine spread and/or recurrence. The tumors are composed of spindled cells. The main differentials include smooth muscle tumors and endometrial stromal tumors and their morphology may overlap with that of UIMT. Immunohistochemical positivity for ALK or FISH testing for ALK rearrangements can help in the diagnosis.

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作者: 张瑜 [1] 董伟 [1] 李淑霞 [1] 赵晶晶 [1] 杜彬 [1]
期刊: 《中华病理学杂志》2021年50卷9期 1020-1023页 MEDLINEISTICPKUCSCD
栏目名称: 论著
DOI: 10.3760/cma.j.cn112151-20210110-00024
发布时间: 2022-06-15
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