95例肾母细胞瘤临床病理特点及预后因素分析

  目的  分析肾母细胞瘤临床病理特点及预后相关因素。  方法  对首都医科大学附属北京儿童医院2001年1月至2007年12月期间收治的95例肾母细胞瘤资料进行收集, 分析其预后相关因素。  结果  本组研究中, 男性61例(60%), 平均发病年龄为3.16岁, 最常见的症状为腹部包块, 92例接受了手术治疗, 其中88例行完整肾肿瘤切除。Ⅰ期病例占41%、Ⅲ期为25%。随访患儿的总体生存率为78.9%, 随访的中位时间间隔为76.7个月, 分期是与预后相关的主要因素; 1例马蹄肾肾母细胞瘤; 7例患儿合并其他畸形。  结论  本组病例85%的肾母细胞瘤患儿于5岁前起病, 常见的症状为腹部包块, 右侧略多于左侧。以Ⅰ、Ⅲ期为主, 病理分型以FH为主, 转移部位为肺和肝脏。肾母细胞瘤总体生存率为78.9%, 分期是与预后相关的主要因素。特殊类型的肾母细胞瘤为马蹄肾肾母细胞瘤, 可并发其他器官先天畸形。 

Analysis of the Clinicopathologic Characteristics and Prognostic Factors of Nephroblastoma

  Objectives  To analyze the clinicopathologic features and the prognostic factors of nephroblastomas (Wllms' tumor).  Methods  A database was established for nephroblastoma cases admitted from January 200l to December 2007 in the Department of Surgical Oncology, Beijing Children's Hospital.  Results  A sample of A total of 61 boys (60%) were enrolled into the study. The median age was 3.16 years. The most common symptom was abdominal mass. Among the 92 patients with surgical treatment, 88 cases underwent typical nephrectomy. The clinical staging of the patients were as follows: 37 cases (41%) were in stage I, 22 cases (25%) were in stage III. Histopathology indicated 76 cases with favorable histology (FH) and 1 case had an unfavorable histology (UFH). The overall survival rate was 78.9%. The median follow-up time was 76.7 months. Staging was the most important factor associated with prognosis. One case of horseshoe kidney and seven cases of other congenital malformation were reported.  Conclusion  Up to 85% of the patients reported an onset before 5 years old. The most common symptom was abdominal mass. The right side of the kidney is more commonly involved than the left. Stage 1 and III were more common among the study population. FH is the major type in histopathology. The most common metastatic sites were the lungs and liver. In this group, the overall survival rate was 78.9%. Staging was the main prognostic factor. The special type of Wilms' tumor in a horseshoe-kidney. Some malformation were also associated with nephroblastoma.