van der Beek Justine N, Fitski Matthijs, de Krijger Ronald R, Vermeulen Marijn A, Nikkels Peter G J, Maat Arie, Buser Myrthe A D, Wijnen Marc H W A, Hendrikse Jeroen, van den Heuvel-Eibrink Marry M, van der Steeg Alida F W, Littooij Annemieke S
Princess Máxima Center for Pediatric Oncology, Utrecht, The Netherlands.
Department of Radiology and Nuclear Medicine, University Medical Center Utrecht/Wilhelmina Children's Hospital, Utrecht, The Netherlands.
Eur Radiol. 2025 Feb;35(2):652-663. doi: 10.1007/s00330-024-10959-2. Epub 2024 Aug 8.
The International Society of Paediatric Oncology-Renal Tumour Study Group (SIOP-RTSG) discourages invasive procedures to determine the histology of paediatric renal neoplasms at diagnosis. Therefore, the histological subtype of Wilms' tumours (WT) is unknown at the start of neoadjuvant chemotherapy. MR-DWI shows potential value as a non-invasive biomarker through apparent diffusion coefficients (ADCs). This study aimed to describe MR characteristics and ADC values of paediatric renal tumours to differentiate subtypes.
Children with a renal tumour undergoing surgery within the SIOP-RTSG 2016-UMBRELLA protocol were prospectively included between May 2021 and 2023. In the case of a total nephrectomy, a patient-specific cutting guide based on the neoadjuvant MR was 3D-printed, allowing a correlation between imaging and histopathology. Whole-tumour volumes and ADC values were statistically compared with the Mann-Whitney U-test. Direct correlation on the microscopic slide level was analysed through mixed model analysis.
Fifty-nine lesions of 54 patients (58% male, median age 3.0 years (range 0-17.7 years)) were included. Forty-four lesions involved a WT. Stromal type WT showed the lowest median decrease in volume after neoadjuvant chemotherapy (48.1 cm, range 561.5-(+)332.7 cm, p = 0.035). On a microscopic slide level (n = 240 slides) after direct correlation through the cutting guide, stromal areas showed a significantly higher median ADC value compared to epithelial and blastemal foci (p < 0.001). With a cut-off value of 1.195 * 10 mm/s, sensitivity, and specificity were 95.2% (95% confidence interval 87.6-98.4%) and 90.5% (95% confidence interval 68.2-98.3%), respectively.
Correlation between histopathology and MR-DWI through a patient-specific 3D-printed cutting guide resulted in significant discrimination of stromal type WT from epithelial and blastemal subtypes.
Stromal Wilms' tumours could be discriminated from epithelial- and blastemal lesions based on high apparent diffusion coefficient values and limited decrease in volume after neoadjuvant chemotherapy. This may aid in future decision-making, especially concerning discrimination between low- and high-risk neoplasms.
MR-DWI shows potential value as a non-invasive biomarker in paediatric renal tumours. The patient-specific cutting guide leads to a correlation between apparent diffusion coefficient values and Wilms' tumour subtype. Stromal areas could be discriminated from epithelial and blastemal foci in Wilms' tumours based on apparent diffusion coefficient values.
国际小儿肿瘤学会肾肿瘤研究组（SIOP - RTSG）不主张在诊断时采用侵入性程序来确定小儿肾肿瘤的组织学类型。因此，在新辅助化疗开始时，威尔姆斯瘤（WT）的组织学亚型尚不清楚。磁共振扩散加权成像（MR - DWI）通过表观扩散系数（ADC）显示出作为一种非侵入性生物标志物的潜在价值。本研究旨在描述小儿肾肿瘤的磁共振特征和ADC值以区分亚型。
2021年5月至2023年期间，前瞻性纳入了在SIOP - RTSG 2016 - UMBRELLA方案下接受手术的肾肿瘤患儿。在全肾切除术的情况下，基于新辅助磁共振成像3D打印患者特异性切割导板，从而实现成像与组织病理学之间的关联。采用曼 - 惠特尼U检验对全肿瘤体积和ADC值进行统计学比较。通过混合模型分析在显微镜载玻片水平上进行直接相关性分析。
纳入了54例患者的59个病灶（58%为男性，中位年龄3.0岁（范围0 - 17.7岁））。44个病灶为WT。间质型WT在新辅助化疗后体积的中位减少量最低（48.1 cm，范围561.5 -（+）332.7 cm，p = 0.035）。通过切割导板进行直接关联后，在显微镜载玻片水平（n = 240张载玻片）上，间质区域的ADC值中位数显著高于上皮和胚芽灶（p < 0.001）。以1.195×10⁻³mm²/s为临界值，敏感性和特异性分别为95.2%（95%置信区间87.6 - 98.4%）和90.5%（95%置信区间68.2 - 98.3%）。
通过患者特异性3D打印切割导板实现组织病理学与MR - DWI之间的关联，能够显著区分间质型WT与上皮和胚芽亚型。
基于高表观扩散系数值和新辅助化疗后体积减少有限，间质型威尔姆斯瘤可与上皮和胚芽性病变相鉴别。这可能有助于未来的决策制定，特别是在区分低风险和高风险肿瘤方面。
MR - DWI在小儿肾肿瘤中显示出作为非侵入性生物标志物的潜在价值。患者特异性切割导板导致表观扩散系数值与威尔姆斯瘤亚型之间的关联。基于表观扩散系数值，威尔姆斯瘤的间质区域可与上皮和胚芽灶相鉴别。
