目的：探讨儿童特殊类型肾母细胞瘤的临床病理学特点、诊断要点及鉴别诊断。方法：收集5例儿童特殊类型肾母细胞瘤病例进行临床资料、病理形态学及免疫组织化学分析，并结合文献探讨其诊断与鉴别诊断。结果：5例患儿中男童3例，女童2例，年龄4月~2岁，中位年龄1岁，主因腹部包块、无痛性肉眼血尿或腹胀、发热就诊，左肾(3例)多于右肾(2例)。其中部分囊状分化型3例，镜下见肿瘤由大小不一的囊腔构成，内衬扁平、立方或鞋钉样上皮细胞，间隔见间叶、胚芽或上皮的肾母细胞瘤成分；肾母细胞瘤伴肾源性残余2例，镜下见由间叶、胚芽和上皮等构成的典型肾母细胞瘤成分，周围见胚芽和上皮细胞与正常肾组织混合存在。结论：部分囊状分化的肾母细胞瘤(cystic partially differentiated nephroblastoma，CPDN)、肾母细胞瘤伴肾源性残余是少见特殊类型的肾母细胞瘤，术前临床诊断及鉴别诊断困难，只有依靠术后病理作出准确诊断，为患儿治疗及预后提供可靠依据。
Special types of Wilms’ tumor: a clinicopathological analysis of 5 cases
Objective: To explore the clinicopathological features, pathological diagnosis and differential diagnosis of special types of Wilms’ tumor. Methods: Five cases of special Wilms’ tumor were studied with analysis of clinical data, pathological and immunohistochemical features, and the diagnosis and differential diagnosis were discussed with review of literatures. Results: There were 3 boys and 2 girls with age ranging from 4 months to 2 years old (median 1 year old). The clinical manifestations were abdominal mass, painless gross hematuria, bloating or fever. Tumors located in the left kidney more than right kidney. Microscopic examination showed cysts with different sizes and lined with flat, cubic or hobnail epithelial cells, between them there were Wilms’ tumor components, such as mesenchymal, germ or epithelium in 3 cases of cystic partially differentiated nephroblastoma. The other 2 cases were composed of classical Wilms’ tumor and nephrogenic rests which mean germ and epithelial cells scattered in normal kidney. Conclusion: Cystic partially differentiated nephroblastoma and Wilms’ tumor with nephrogenic rests were rare and special types of Wilms’ tumor. Because of difficulty of diagnosis and differential diagnosis before operation, the pathological diagnosis is the most accurate method and the most reliable accordance for treatment and prognosis.