小儿肾透明细胞肉瘤及肾恶性横纹肌样瘤临床特征和CT表现  

The CT Manifestations and Clinical Characteristics of Clear Cell Sarcoma and Malignant Rhabdoid Tumor of the Kidney in Children

作　　者：徐守军[1] 杨春兰[2] 曹娟[3] 谢丽春 刘龙平 裴妮慧 罗娜[1] 刘劼 XU Shoujun;YANG Chunan;CAO Juan(Department of Radiology,Shenzhen Children's Hospital,Shenzhen,Guangdong Province 518026,P.R.China)

机构地区：[1]深圳市儿童医院放射科,518026 [2]深圳市儿童医院血液肿瘤科,518026 [3]深圳市儿童医院病理科,518026 [4]广州医科大学附属第三医院儿科,510530

出　　处：《临床放射学杂志》2024年第5期811-818,共8页Journal of Clinical Radiology

基　　金：广东省高水平临床重点专科项目(编号:SZGSP012);广东省基础与应用基础研究基金项目(编号:2022A1515111121);深圳市医学重点学科建设经费(编号:SZXK034);深圳市医疗卫生三名工程项目(编号:SZSM202011005);深圳市基础研究专项项目(自然科学基金)基础研究面上项目资助(编号:JCYJ20220530155616038)。

摘　　要：目的 探讨小儿肾透明细胞肉瘤(CCSK)和肾恶性横纹肌样瘤(MRTK)的临床特征和CT表现。方法回顾性分析4例CCSK和4例MRTK患儿临床资料。8例术前均行CT平扫及增强扫描。结果 4例CCSK,左肾1例,右肾3例;4例MRTK,左肾3例,右肾1例。病灶均呈类圆形。瘤体较大,CCSK病灶最大径线为52~120 mm,平均(81±25)mm;MRTK病灶最大径线为41~81 mm,平均(62±15)mm。4例CCSK主要表现为残肾形态存在,肾组织环绕病灶;3例病灶位于肾盂内,肾盂变形、扩大,1例突破肾盂向外生长;CT平扫呈等或稍低密度影,出血较多时可表现为不均匀稍高密度影。增强扫描轻-中度不均匀渐进性强化。4例均见坏死/囊变及出血;1例见包膜下积液/血,即“新月征”;未见钙化及骨质破坏;1例肺及淋巴结转移。4例MRTK中3例残肾形态消失,仅1例存在;残肾肾盂均受压、推移;病灶为囊实性,分界不清,呈渐变性改变,表现为“融冰征”。增强扫描轻-中度不均匀渐进性强化。4例病灶均有坏死/囊变;3例内见高密度出血影;2例含有钙化;4例出现“新月征”;均未见骨质破坏;1例脑转移,1例肺及淋巴结转移,1例下腔静脉及左肾静脉见瘤栓。结论 小儿CCSK和MRTK发病率较低,CT表现与小儿其他常见肾恶性肿瘤具有许多相似性,以及对其征象认识不足是误诊的主要原因。全面了解CCSK及MRTK的影像学表现,分析误诊原因,有助于准确诊断。Objective To investigate the imaging and clinical characteristics of clear cell sarcoma of the Kidney(CCSK) and malignant rhabdoid tumor of the kidney in children. Methods We conducted a retrospective analysis the case data of the 4 children with CCSK and 4 children with MRTK confirmed by pathologically.All 8 cases were examined with plain and enhanced CT scans preoperative. Results 4 cases of CCSK,1 was located in the left kidney and 3 in right kidney.4 cases of MRTK,3 were located in the left kidney, 1 was in right kidney. The 8 lesions were all circular. The tumors were all large, and the maximum diameter of the CCSK lesions ranged from 34 mm to 149 mm, with an average of(73±35)mm. The maximum diameter of MRTK lesions ranged from 41 mm to 81 mm, with an average of(62±15)mm.4 cases of CCSK mainly showed residual kidney morphology, kidney tissue around the lesion. The lesion was located in the renal pelvis with deformation and enlargement in 3 cases, and broke through the renal pelvis and grew outward in 1 case. The main findings showed equal or slightly low density mass, but uneven slightly high density mass with more bleeding no Non-enhanced CT. Showed mild to moderate uneven progressive enhancement after contrast—enhanced scanning. Necrosis/cystic change and hemorrhage were observed in all 4 cases. Subcapsular effusion/blood was observed in 1 case, namely “crescent sign”;No calcification or bone destruction was observed. Lung and lymph node metastases in 1 case. Among the 4 cases of MRTK,there is dual kidney disappeared in 3 cases and only 1 existed. Residual renal pelvis were pressed and pushed. The lesions were heterogeneous solid mass, mostly with cystic degeneration, and the boundary between cystic and solid was not clear, Characterized by changing gradually on Non-enhanced CT,manifested as “melting ice sign”. Showed mild to moderate uneven progressive enhancement after contrast—enhanced scanning. There was necrosis/cystic change in all the 4 cases.3 cases showed high density bleeding shadow

关 键 词：肾透明细胞肉瘤 肾恶性横纹肌样瘤 体层摄影术 X线计算机 误诊分析 

分 类 号：R737.11[医药卫生—肿瘤] R730.44[医药卫生—临床医学]