Abstract：
Non-invasive differentiation of paediatric kidney tumours is particularly important in the SIOP-RTSG protocols, which recommend pre-operative chemotherapy without histological confirmation. The identification of clinical and tumour-related parameters may enhance diagnostic accuracy. Age, metastases, and tumour volume (TV) were retrospectively analysed in 3306 patients enrolled in SIOP/GPOH 9, 93-01, and 2001 including Wilms tumour (WT), congenital mesoblastic nephroma (CMN), clear cell sarcoma (CCSK), malignant rhabdoid tumour of the kidney (MRTK), and renal cell carcinoma (RCC). WT was diagnosed in 2927 (88.5%) patients followed by CMN 138 (4.2%), CCSK 126 (3.8%), MRTK 58 (1.8%) and RCC 57 (1.7%). CMN, the most common localized tumour (71.6%) in patients younger than 3 months of age, was diagnosed earliest and RCC the latest (median age [months]: 0 and 154, respectively) both associated with significantly smaller TV (median TV [mL]: 67.2 and 45.0, respectively). RCC occurred in >14% of patients older than 120 months or older than 84 months with TV <100 mL. Receiver operating characteristic analyses discriminated WT from CMN, RCC and MRTK regarding age (AUC = 0.976, 0.929 and 0.791) and TV (AUC = 0.768, 0.813 and 0.622). MRTK had the highest risk of metastasis (37.9%) despite young age, whereas the risk of metastasis increased significantly with age in WT. Age and TV at diagnosis can differentiate WT from CMN and RCC. MRTK must be considered for metastatic tumours at young age. Identification of CCSK without histology remains challenging. Combined with MRI-characteristics, including diffusion-weighted imaging, and radiomics and liquid biopsies in the future, our approach allows optimization of biopsy recommendations and prevention of misdiagnosis-based neoadjuvant treatment.
摘要：
儿科肾脏肿瘤的非侵入性分化在SIOP-RTSG方案中尤为重要。建议术前化疗，无需组织学证实。临床和肿瘤相关参数的识别可以提高诊断准确性。年龄,转移,和肿瘤体积（TV）回顾性分析了3306例SIOP/GPOH9，93-01和2001，包括Wilms肿瘤（WT），先天性中胚层肾瘤（CMN），透明细胞肉瘤(CCSK),肾脏恶性横纹肌样瘤（MRTK），和肾细胞癌（RCC）。在2927例（88.5%）患者中诊断出WT，其次是CMN138例（4.2%），CCSK126（3.8%），MRTK58（1.8%）和RCC57（1.7%）。CMN,3个月以下患者中最常见的局部肿瘤（71.6％），最早诊断为RCC，最晚诊断为RCC（中位年龄[月]：分别为0和154），均与显着较小的TV（中位TV[mL]：分别为67.2和45.0）相关。在大于120个月或大于84个月且电视<100mL的患者中，>14%的患者发生RCC。接收机工作特性分析区分WT和CMN，RCC和MRTK关于年龄(AUC=0.976、0.929和0.791)和电视(AUC=0.768、0.813和0.622)。尽管年龄较小，但MRTK的转移风险最高（37.9％）。而WT的转移风险随着年龄的增长而显著增加。诊断时的年龄和电视可以将WT与CMN和RCC区分开。MRTK必须在年轻时考虑转移性肿瘤。没有组织学的CCSK鉴定仍然具有挑战性。结合MRI特征，包括弥散加权成像，未来的影像组学和液体活检，我们的方法可以优化活检建议和预防基于误诊的新辅助治疗.
