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作 者:龚春香 邵馨[1] 范钦和 Gong Chunxiang;Shao Xin;Fan Qinhe(Department of Pathology,Changzhou Traditional Chinese Medicine Hospital Affiliated to Nanjing University of Chinese Medicine,Changzhou 213003,Jiangsu,China;Department of Pathology,the First Affiliated Hospital of Nanjing Medical University(Jiangsu Province Hospital),Nanjing 210029,China)
机构地区:[1]南京中医药大学附属常州市中医医院病理科,常州213003 [2]南京医科大学第一附属医院病理学部,南京210029
出 处:《中华皮肤科杂志》2023年第12期1158-1162,共5页Chinese Journal of Dermatology
摘 要:目的探讨浅表性CD34阳性成纤维细胞肿瘤(SCPFT)临床、组织病理、免疫表型、分子遗传特征。方法回顾性分析南京医科大学第一附属医院病理科2015年1月至2022年7月确诊的19例SCPFT的病理特征。结果纳入19例SCPFT患者,男8例,女11例,发病年龄16~67岁,病程3个月至3年。肿瘤发生在大腿6例、背部4例、肩部3例、腹壁2例、腘窝1例、小腿1例、足底1例、额头1例。肿瘤长径0.8~4(2.36±0.76)cm,境界相对清楚。19例均经手术切除,1例复发。组织病理检查:肿瘤位于真皮及皮下组织,可向周围脂肪组织或真皮浸润;肿瘤细胞丰富,由梭形、多边形细胞交织状、束状排列;所有病例均可见多形性细胞,细胞核大,深染,不规则,可见核内假包涵体,常见显著核仁;核分裂象罕见,未见肿瘤性坏死;肿瘤间质内有不同程度炎症细胞浸润,其中2例肿瘤周围见淋巴细胞鞘。免疫组化染色:19例肿瘤细胞弥漫强阳性表达CD34,18例增殖指数Ki-67低于10%,少数病例局灶表达CKpan(2/12)、结蛋白(2/15)、平滑肌肌动蛋白(2/19)。荧光原位杂交检测3例PRDM10基因重排情况,2例阳性,其中1例伴有淋巴细胞鞘的SCPFT无EWSR1基因重排。结论SCPFT是一种中间性成纤维细胞性肿瘤,常伴PRDM10基因重排,临床无特征性表现,诊断依赖组织病理形态。Objective To investigate clinical,histopathological,molecular genetic characteristics,and immunophenotypes of superficial CD34-positive fibroblastic tumor(SCPFT).Methods The pathological data were collected from 19 cases of SCPFT from January 2015 to July 2022 in the First Affiliated Hospital of Nanjing Medical University,and analyzed retrospectively.Results In total,19 cases of SCPET were enrolled,including 8 males and 11 females.Their age at onset ranged from 16 to 67 years,and the disease duration ranged from 3 months to 3 years.Tumors occurred in the thigh(6 cases),back(4 cases),shoulder(3 cases),abdominal wall(2 cases),popliteal fossa(1 case),lower leg(1 case),foot(1 case)and forehead(1 case).The tumor lengths were 0.8-4(2.36±0.76)cm,with relatively clear borders.Surgical excision was performed in all the 19 cases,and 1 case experienced recurrence.Histopathological examination showed that tumors were located in the dermis and subcutis,and infiltrated the peripheral fat tissues or the dermis;tumors were composed of abundant spindle cells and polygonal cells arranged in interlacing fascicles;cells with nuclear pleomorphism were observed in all cases,and were characterized by large,hyperchromatic and irregular nuclei,intranuclear pseudoinclusions,and prominent nucleoli;the mitotic figure was rare,and tumor necrosis was not found;inflammatory cell infiltration to different extents was observed in the tumor stroma,and lymphocyte sheaths were observed around the tumors in 2 cases.Immunohistochemical staining showed strong diffuse expression of CD34 in the tumor cells in all the 19 cases,the Ki-67 proliferation index was<10%in 18 cases,and CKpan(2/12),desmin(2/15),and smooth muscle actin(1/19)were focally expressed.Fluorescence in situ hybridization for the detection of PRDM10 gene rearrangements was performed in 3 cases,and identified PRDM10 rearrangements in 2 cases,including 1 case of SCPFT accompanied by lymphocyte sheaths without EWSR1 rearrangements.Conclusion SCPFT is an intermediate fibroblastic tumor with
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