儿童囊性部分分化型肾母细胞瘤的影像特征分析及文献复习OA
Imaging features and literature review of cystic partially differentiated nephroblastoma in children
目的:探讨儿童囊性部分分化型肾母细胞瘤(Cystic partially differentiated nephroblastoma,CPDN)的影像特征.方法:回顾性分析我院8例儿童CPDN的临床、病理及影像资料,7例行超声检查,8例行CT平扫及增强扫描,2例行MRI平扫及增强扫描,并结合文献总结CPDN的影像特征.结果:女6例,男2例,年龄5月~3岁(中位年龄1岁3月),临床表现为腹部膨隆、触及腹部肿物、血尿等.常规型7例,属Bosniak Ⅲ级;乳头结节型1例,属Bosniak Ⅳ级.肿瘤囊隔中位最大厚度7.69 mm,增强扫描多呈轻-中度强化.乳头结节型病例结节长径1.81 cm,增强扫描轻度强化.囊腔中位最大长径4.14 cm.囊液在超声上表现为低或无回声,CT值范围3.63~29.93 HU,MRI呈T1WI低或稍低信号、T2WI高信号,合并囊内出血及左肾上盏扩张积液各1例.肿物大体由多发囊隔及囊腔组成.根据镜下囊隔内高分化组织占比,7例常规型属1级,1例乳头结节型属2级,囊壁覆以立方或鞋钉状上皮,囊隔及结节内含一定数量不成熟的肾胚芽、上皮性小管或肾小球结构及间叶组织成分.结论:儿童CPDN影像表现多样且具有一定特征.常规型不伴实性结节,乳头结节型为CPDN特殊表型.囊隔较密集、较厚的区域往往伴有较小囊腔,囊隔较稀疏、较薄的区域往往伴有较大囊腔,囊液的影像学表现反映其内部成分差异.
Objective:To investigate the imaging features of cystic partially differentiated nephroblastoma(CPDN)in chil-dren.Methods:The clinical,pathological,and imaging data of eight pediatric CPDN cases treated at our institution were retrospectively analyzed.Ultrasonography was performed in seven cases;all eight cases underwent non-contrast and contrast-enhanced CT,and two cases underwent non-contrast and contrast-enhanced MRI.The imaging features of CPDN were sum-marized in combination with relevant literature.Results:The cohort included six females and two males,aged 5 months to 3 years(median age:1 year 3 months).Clinical manifestations included abdominal distension,palpable abdominal mass,and hematuria.Seven cases were of the conventional type and classified as Bosniak Ⅲ;one case was of the papillonodular type and classified as Bosniak Ⅳ.The median maximal thickness of the septa was 7.69 mm,with predominantly mild to moder-ate enhancement.In the papillonodular case,the nodular component measured 1.81 cm in maximal diameter and showed mild enhancement.The median maximal diameter of the cystic cavity was 4.14 cm.Cystic fluid appeared anechoic or hypoechoic on ultrasonography,had CT attenuation values ranging from 3.63 to 29.93 HU,and exhibited low or slightly low signal on T1WI and high signal on T2WI.One case was complicated by intracystic hemorrhage and another by left upper calyceal dilatation with hydronephrosis.The tumors were generally composed of multiple septa and cystic spaces.Based on the proportion of well-differentiated tissue within the septa,seven conventional-type cases were classified as grade 1,and the papillonodular case as grade 2.The cyst walls were lined by cuboidal or hobnail epithelium,the septa and nodules con-tained a certain amount of immature renal germ,epithelial tubules or glomeruloid structures,and mesenchymal components.Conclusion:Pediatric CPDN has diverse imaging manifestations with certain characteristic features.Conventional CPDN lacks solid nodules,while papillonodular CPDN is a specific variant.Areas with denser and thicker septa were usually asso-ciated with small cysts,whereas areas with thinner and sparser septa tender to contain large cysts,and the imaging charac-teristics of the cystic fluid reflect differences in its internal composition.
陈希文;张芊韵;袁理;陈臻;吴慧莹;鹿连伟;唐清梅
广州医科大学附属妇女儿童医疗中心,广东 广州 510623广州医科大学附属妇女儿童医疗中心,广东 广州 510623广州医科大学附属妇女儿童医疗中心,广东 广州 510623广州医科大学附属妇女儿童医疗中心,广东 广州 510623广州医科大学附属妇女儿童医疗中心,广东 广州 510623广州医科大学附属妇女儿童医疗中心,广东 广州 510623广州医科大学附属妇女儿童医疗中心,广东 广州 510623
医药卫生
Wilms瘤儿童体层摄影术,X线计算机磁共振成像
Wilms TumorChildTomography,X-Ray ComputedMagnetic Resonance Imaging
《中国临床医学影像杂志》 2026 (9)
654-657,4
广东省医学科学技术研究基金(A20211261).
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