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[膀胱炎性肌纤维母细胞瘤:10例临床病理研究]

[Inflammatory myofibroblastic tumors of the urinary bladder: a clinicopathological study of ten cases].

作者信息

Zhang H Z, Wang S Y, Dong L, Yang X Q, Wang C F

机构信息

Department of Pathology, Ningbo Diagnostic Pathology Center, Zhejiang Province, Ningbo 315000, China.

Department of Pathology, Rui Jin Hospital, School of Medicine, Shanghai Jiaotong University, Shanghai 200020, China.

出版信息

Zhonghua Bing Li Xue Za Zhi. 2021 Sep 8;50(9):1024-1028. doi: 10.3760/cma.j.cn112151-20210113-00039.

Abstract

To investigate the clinicopathological features,immunohistochemical phenotypes, molecular genetic alterations,diagnosis and differential diagnosis of inflammatory myofibroblastic tumor (IMT) of the urinary bladder. Ten cases of IMT of the urinary bladder (three cases at Ningbo Diagnostic Pathology Center from September 2011 to December 2020, five in-house diagnosed cases and two consultation cases at Shanghai Rui Jin Hospital from June 2011 to December 2020) were collected retrospectively. The clinicopathologic features and immunophenotypic profiles were studied by light microscopy and immunohistochemistry (EnVision method). The translocation of ALK gene was detected by fluorescence in situ hybridization (FISH). Of the 10 patients, eight were male and two were female. The patients' age range was 16 to 62 years (median 36 years).The main clinical presentation was hematuria and urinary irritation. Three cases were located at the dome of the urinary bladder, four cases were in the left lateral wall and the remaining three cases were in the right lateral wall. The tumor size ranged from 1.5 cm to 8.5 cm. In eight cases, the tumors were mainly submucosal, and in some cases extending to the muscular layer of the urinary bladder. In two cases, the tumors were mainly located in the muscular layer and focally extended to the submucosa and adventitia. Histologically, four cases had the nodular fasciitis-like pattern, three cases had fibrohistiocytoma-like pattern, two cases had mixed histologic patterns and the remaining case showed leiomyosarcoma-like histologic features. Immunohistochemically, the tumor cells expressed SMA (10/10),calponin (9/10),desmin (6/10) and CKpan (9/10). Cytoplasmic staining for ALK1 and ALK (5A4) was detected in 7 of 10 cases and 8 of 10 cases, respectively. Nuclear and cytoplasmic staining for ALK (D5F3) was detected in 7 of 10 cases. Among eight cases with material available for FISH analysis, ALK rearrangement was present in five cases. Follow-up data were available in eight patients and none had local recurrence nor distant metastasis. IMT of the urinary bladder is an uncommon mesenchymal neoplasm with intermediate malignant potential.It has special clinicopathologic features, and a minority of cases have local tumor recurrence.

摘要

探讨膀胱炎性肌纤维母细胞瘤(IMT)的临床病理特征、免疫组化表型、分子遗传学改变、诊断及鉴别诊断。回顾性收集10例膀胱IMT(2011年9月至2020年12月宁波诊断病理中心3例,2011年6月至2020年12月本院确诊5例及上海瑞金医院会诊2例)。通过光学显微镜和免疫组化(EnVision法)研究其临床病理特征和免疫表型。采用荧光原位杂交(FISH)检测ALK基因易位。10例患者中,男性8例,女性2例。患者年龄范围为16至62岁(中位年龄36岁)。主要临床表现为血尿和尿路刺激症状。3例位于膀胱顶部,4例位于左侧壁,其余3例位于右侧壁。肿瘤大小为1.5 cm至8.5 cm。8例肿瘤主要位于黏膜下层,部分病例延伸至膀胱肌层。2例肿瘤主要位于肌层,局部延伸至黏膜下层和外膜。组织学上,4例呈结节性筋膜炎样形态,3例呈纤维组织细胞瘤样形态,2例为混合组织学形态,其余1例表现为平滑肌肉瘤样组织学特征。免疫组化显示,肿瘤细胞表达SMA(10/10)、钙调蛋白(9/10)、结蛋白(6/10)和CKpan(9/10)。10例中有7例和8例分别检测到ALK1和ALK(5A4)的胞质染色。10例中有7例检测到ALK(D5F3)的核和胞质染色。在8例可进行FISH分析的病例中,5例存在ALK重排。8例患者有随访资料,均无局部复发及远处转移。膀胱IMT是一种罕见的间叶性肿瘤,具有中等恶性潜能。它具有特殊的临床病理特征,少数病例有局部肿瘤复发。

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