儿童肾透明细胞肉瘤五例临床病理分析

Clear cell sarcoma of the kidney in children:a clinicopathological analysis of 5 cases

目的探讨儿童肾透明细胞肉瘤(CCSK)的临床病理学特征、诊断及鉴别诊断。方法回顾性分析2019—2022年于郑州市第一人民医院及河南省儿童医院病理科诊断的5例CCSK患儿的临床特征、病理形态、免疫表型,并复习相关文献。结果5例患儿中,男4例,女1例,其中4例1~3岁,1例为4岁6个月,影像学检查均提示肾脏肿块。镜下见肿瘤细胞弥漫增生,呈片状、条索状或巢团状排列,细胞呈圆形、卵圆形或短梭形,大小一致,细胞质淡染或透明,细胞核圆形或卵圆形,染色质细腻,核仁不明显,核分裂像少见,间质纤维组织内有较多薄壁血管。免疫表型:5例CyclinD1、BCOR、INI-1、Vimentin均阳性,4例Bcl-2阳性,3例CD99阳性,5例CK、EMA、S-100、WT-1、HMB-45、Desmin均阴性,Ki-67增殖指数10%~50%阳性。结论CCSK主要发生于儿童肾脏,发病率较低,容易漏诊和误诊,诊断时需结合临床特征、病理组织形态及免疫组化等资料进行全面分析。

Objective To investigate the clinicopathological characteristics,diagnosis method and differential diagnosis method of clear cell sarcoma of the kidney(CCSK)in children.Methods The clinical features,pathological morphology,and immunophenotype of 5 children with clear cell sarcoma of the kidney(CCSK)diagnosed in Department of Pathology of the First People’s Hospital of Zhengzhou and Henan Children’s Hospital from 2019 to 2022 were retrospectively analyzed.And relevant literature was reviewed.Results Among the 5 children,there were 4 boys and 1 girl,4 cases aged 1-3 years,and 1 case aged 4 years and 6 months;the imaging examination results of the 5 children all displayed renal tumor.Microscopically,the tumor cells were diffusely hyperplastic,arranged as sheets,cords or nests,the cytoplasm was light stained or transparent,the nuclei were circular or ovoid,the chromatin was delicate,the nucleoli were not obvious,and nuclear division were rare,there were many thin-walled blood vessels in the interstitial fibrous tissue.Results of immunophenotype showed that CyclinD1,BCOR,INI-1,and Vimentin were positive in the 5 children,Bcl-2 was positive in 4 children,and CD99 was positive in 3 children;while CK,EMA,S-100,WT-1,HMB-45 and Desmin were negative in the 5 children.Ki-67 proliferation index was 10%-50%in all children.Conclusions CCSK primarily occurs in children kidney with a low morbidity,but it is easy to be missed diagnosis and misdiagnosed.Thus,the detailed clinical characteristic,pathological morphology,and immunophenotype should be comprehensive considered and analyzed during diagnosis.