儿童肾恶性横纹肌样瘤的影像学及临床特点  

Imaging and Clinical Characteristics of Malignant Rhabdoid Tumors of Kidney in Children

作　　者：郑彬 霍亚玲 时胜利 王攀鸽 刘玥[3] ZHENG Bin;HUO Yaling;SHI Shengli;WANG Pange;LIU Yue(Department of Medical Imaging,Zhengzhou Children’s Hospital,Henan Children’s Hospital,Zhengzhou 450000,China;Department of Ultrasound Medicine,Children's Hospital Affiliated to Zhengzhou University,Zhengzhou Children’s Hospital,Henan Children’s Hospital,Zhengzhou 450000,China;Imaging Center,Beijing Children’s Hospital,Capital Medical University,National Children’s Medical Center,Beijing 100045,China)

机构地区：[1]郑州大学附属儿童医院,河南省儿童医院郑州儿童医院医学影像科,河南郑州450000 [2]郑州大学附属儿童医院,河南省儿童医院郑州儿童医院超声医学科,河南郑州450000 [3]国家儿童医学中心,首都医科大学附属北京儿童医院影像中心,北京100045

出　　处：《河南医学研究》2024年第9期1540-1545,共6页Henan Medical Research

基　　金：河南省医学科技攻关计划联合共建项目(LHGJ20210668)。

摘　　要：目的探讨儿童肾恶性横纹肌样瘤(MRTK)的影像学及临床特点。方法收集郑州大学附属儿童医院收治的9例MRTK患儿的临床资料,术前均接受CT检查,8例接受超声检查,3例接受MRI检查,总结影像学及临床特点。结果患儿男4例、女5例,中位年龄6个月5天,均单发肿瘤,乳酸脱氢酶(LDH)增高,Ⅱ期~Ⅳ期,发生于左肾6例、右肾3例,临床以腹胀、血尿等症状入院。术前超声提示囊实性或实性为主肿块,肾髓质为中心浸润性生长,与残肾界限不清,其中6例侵犯肾门,1例伴静脉瘤栓。CT平扫密度不均,2例囊实性边界渐变样过渡,2例伴钙化。增强强化不均,强化程度低于周围残肾,其中6例侵犯肾盂,4例伴瘤内出血及肾包膜下积液。肿块MRI信号较混杂,实性部分弥散受限,术前7例伴转移,1例合并颅内肿瘤。病理组织具有大核仁、丰富细胞质的横纹肌样细胞和嗜酸性细胞的包涵体,免疫组化Vimentin(+)9例,EMA(+)4例,CK(+)4例,INI-1(-)6例。6例手术辅以化疗患儿中4例出现复发及转移,2例化疗患儿1例肿块未见缩小并新发淋巴结转移,1例失访,1例放弃治疗患儿死亡。结论MRTK恶性程度高,易转移,预后极差。不同影像学检查方法各有优缺点,MRTK的影像学表现为囊实性或实性为主肿块,浸润性生长,与残肾界限不清,肿块囊实性边界渐变样过渡及肾包膜下积液具有一定特征性。Objective To investigate the imaging and clinical features of malignant rhabdoid tumor of the kidney(MRTK)in children.Methods The clinical data of 9 children with MRTK in Children's Hospital Affiliated to Zhengzhou University were collected,all of which were examined by CT,8 by ultrasound and 3 by MRI before operation.The imaging and clinical features were summarized.Results There were 4 cases of male and 5 cases of female with a median age of 6 months and 5 days,all of them had a single tumor,increased lactate dehydrogenase(LDH),stageⅡ-Ⅳ,6 cases in the left kidney and 3 cases in the right kidney,and were hospitalized with symptoms such as abdominal distension and hematuria.Preoperative ultrasonography revealed a cystic or solid mass with central invasive medullary growth and unclear boundary with the residual kidney.There were 6 cases of renal hilar invasion and 1 case with venous tumor thrombus.CT plain scan density was uneven,2 cases had cystic solid boundary transition,2 cases with calcification.The degree of enhancement was less than that of the peripheral residual kidney.Six cases involved the renal pelvis and 4 cases were accompanied by intratumoral hemorrhage and subcapsular fluid.MRI signals were mixed and solid partial diffusion was limited.Seven cases were accompanied by metastasis before surgery,and 1 case was accompanied by intracranial tumor.The pathological tissue was composed of large nucleolus,cytoplasmic rich striated muscle cells and eosinophilic cells.Immunohistochemical Vimentin(+)in 9 cases,EMA(+)in 4 cases,CK(+)in 4 cases,and INI-1(-)in 6 cases.Among the 6 children with surgery and chemotherapy,4 patients had recurrence and metastasis,2 patients with chemotherapy had no reduction of the mass and new lymph node metastasis,1 patient was lost to follow-up,and 1 child gave up treatment and died.Conclusion MRTK has high malignant degree,easy metastasis and poor prognosis.Different imaging methods have their advantages and disadvantages.The imaging findings of MRTK are cystic or solid primary

关 键 词：儿童 肾脏 恶性横纹肌样瘤 影像学表现 临床特点 

分 类 号：R445[医药卫生—影像医学与核医学]