Miniati Doug, Gay Andre N, Parks Kathleen V, Naik-Mathuria Bindi J, Hicks John, Nuchtern Jed G, Cass Darrell L, Olutoye Oluyinka O
Division of Pediatric Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, TX, USA.
J Pediatr Surg. 2008 Jul;43(7):1301-7. doi: 10.1016/j.jpedsurg.2008.02.077.
The purpose of this study is to determine the actual incidence, age distribution, and preoperative imaging accuracy of non-Wilms' tumors (nWT) in children with renal masses.
Pathologic reports from all tumor nephrectomies or open renal biopsies performed at a single institution from September 1999 to June 2005 were analyzed. Patient demographics, pathologic findings, specific imaging study descriptors, and differential diagnoses were tabulated. Accuracy of imaging studies in identifying specific tumors was calculated.
Ninety-two patients were identified. Sixty-eight had Wilms' tumor (WT) and 24 had an nWT. The nWT group included congenital mesoblastic nephroma (5), clear cell sarcoma (4), neuroblastoma (4), renal cell carcinoma (4), lymphoma (2), angiomyolipoma (2), teratoma (1), hemangioma (1), and renal epithelial tumor (1). When grouped by ages, the incidence of nWT was between 0% and 83%. Sensitivity, specificity, positive predictive value, and negative predictive value for computed tomography (CT) determining a diagnosis of WT were 0.92, 0.55, 0.84, and 0.73, respectively. The CT reports explicitly stated a potential diagnosis in 89% of cases, with a diagnostic accuracy of 82%.
Non-Wilms' tumors may represent a significant proportion of renal tumors in children, especially in children aged less than 6 months or greater than 12 years. Preoperative imaging is of limited value in differentiating these tumors. These data have significant implications for parental counseling, surgical plan, and the choice of neoadjuvant chemotherapy and argue in favor of obtaining a tissue diagnosis before instituting therapy.
本研究旨在确定肾肿块患儿中非威尔姆斯肿瘤(nWT)的实际发病率、年龄分布及术前影像诊断准确性。
分析了1999年9月至2005年6月在单一机构进行的所有肿瘤肾切除术或开放性肾活检的病理报告。将患者人口统计学资料、病理结果、特定影像学检查描述及鉴别诊断制成表格。计算影像学检查对特定肿瘤的诊断准确性。
共确定92例患者。其中68例为威尔姆斯肿瘤(WT),24例为nWT。nWT组包括先天性中胚层肾瘤(5例)、透明细胞肉瘤(4例)、神经母细胞瘤(4例)、肾细胞癌(4例)、淋巴瘤(2例)、血管平滑肌脂肪瘤(2例)、畸胎瘤(1例)、血管瘤(1例)及肾上皮肿瘤(1例)。按年龄分组时,nWT的发病率在0%至83%之间。计算机断层扫描(CT)诊断WT的敏感性、特异性、阳性预测值和阴性预测值分别为0.92、0.55、0.84和0.73。CT报告在89%的病例中明确给出了可能的诊断,诊断准确性为82%。
非威尔姆斯肿瘤可能在儿童肾肿瘤中占相当比例,尤其是6个月以下或12岁以上的儿童。术前影像学检查在鉴别这些肿瘤方面价值有限。这些数据对家长咨询、手术方案及新辅助化疗的选择具有重要意义,支持在开始治疗前获得组织诊断。