Division of Pediatric Surgery, DeWitt Daughtry Family Department of Surgery, University of Miami Miller School of Medicine, Miami, Florida 33136, USA.
J Surg Res. 2010 Oct;163(2):257-63. doi: 10.1016/j.jss.2010.03.061. Epub 2010 Apr 21.
To determine the outcomes and predictors of survival for pediatric non-Wilms renal tumors (NWRT).
The SEER database (1973-2005) was queried for all patients < 20 y of age.
Overall, 349 cases of NWRT were identified. The major histologic groups included renal cell carcinoma (RCC) (44%), clear cell sarcoma of the kidney (CCSK) (17%), and malignant rhabdoid tumor (MRT) (12%). A bimodal age distribution was observed, with tumors commonly presenting in patients ≤4 y of age and ≥15 y of age. More than 50% of RCC presented at ≥15 y of age, whereas ≥80% of CCSK or MRT patients were ≤4 y of age. Most RCC (57%) and CCSK (53%) were locally staged while most MRT presented with distant disease (51%, P < 0.001). Overall 10-y survival was 63% with improved survival observed in patients with CCSK (79%) and RCC (70%) versus MRT (29%, P < 0.001). By univariate analysis, surgical resection was associated with improved overall 10-y survival (68% versus 30%, P < 0.001), while no benefit was observed for radiotherapy (60% versus 63%, P = 0.8). By multivariate analysis, worse overall survival was observed for patients ≥ 10 y old (HR 4.01, P = 0.013) and those with advanced disease (HR = 12.78, P < 0.001). Patients with MRT (HR = 11.61, P < 0.001) and CCSK (HR = 3.68, P = 0.038) had significantly worse prognosis compared with those with RCC. Surgical resection improved overall survival (HR = 0.36, P = 0.001).
For pediatric NWRT, younger patients and those with RCC have improved survival, while a diagnosis of MRT portends a worse prognosis. Surgical extirpation significantly improves survival for all patients.
为了确定儿科非 Wilms 肾肿瘤（NWRT）的生存结果和预测因素。
在 SEER 数据库（1973-2005 年）中查询了所有年龄<20 岁的患者。
总体而言，共发现 349 例 NWRT。主要组织学类型包括肾细胞癌（RCC）（44%）、肾透明细胞肉瘤（CCSK）（17%）和恶性横纹肌样瘤（MRT）（12%）。观察到双峰年龄分布，肿瘤常见于≤4 岁和≥15 岁的患者。超过 50%的 RCC 发生在≥15 岁，而≥80%的 CCSK 或 MRT 患者年龄≤4 岁。大多数 RCC（57%）和 CCSK（53%）为局部分期，而大多数 MRT 为远处疾病（51%，P<0.001）。整体 10 年生存率为 63%，CCSK（79%）和 RCC（70%）的生存率优于 MRT（29%，P<0.001）。单因素分析显示，手术切除与整体 10 年生存率提高相关（68%与 30%，P<0.001），而放疗无获益（60%与 63%，P=0.8）。多因素分析显示，年龄≥10 岁（HR 4.01，P=0.013）和疾病晚期患者（HR=12.78，P<0.001）的总体生存率较差。与 RCC 相比，MRT（HR=11.61，P<0.001）和 CCSK（HR=3.68，P=0.038）患者预后明显较差。手术切除可提高总体生存率（HR=0.36，P=0.001）。
对于儿科 NWRT，年轻患者和 RCC 患者的生存率较高，而 MRT 诊断则预示着预后较差。手术切除可显著提高所有患者的生存率。
