PDF(Pubmed)
Abstract：
Congenital mesoblastic nephroma is the most common solid renal tumor in neonates. Therefore, patients <3 months of age are advised to undergo upfront nephrectomy, whereas invasive procedures at diagnosis in patients ≥3 months of age are discouraged by the International Society of Pediatric Oncology-Renal Tumor Study Group (SIOP-RTSG). Nevertheless, discriminating congenital mesoblastic nephroma, especially from the more common Wilms tumor, solely based on imaging remains difficult. Recently, magnetic resonance imaging (MRI) has become the preferred modality. Studies focusing on MRI characteristics of congenital mesoblastic nephroma are limited.
This study aims to identify diagnostic MRI characteristics of congenital mesoblastic nephroma in the largest series of patients to date.
In this retrospective multicenter study, five SIOP-RTSG national review radiologists identified 52 diagnostic MRIs of histologically proven congenital mesoblastic nephromas. MRI was performed following SIOP-RTSG protocols, while radiologists assessed their national cases using a validated case report form.
Patients (24/52 classic, 11/52 cellular, and 15/52 mixed type congenital mesoblastic nephroma, 2/52 unknown) had a median age of 1 month (range 1 day-3 months). Classic type congenital mesoblastic nephroma appeared homogeneous with a lack of hemorrhage, necrosis and/or cysts, showing a concentric ring sign in 14 (58.3%) patients. Cellular and mixed type congenital mesoblastic nephroma appeared more heterogeneous and were larger (311.6 and 174.2 cm3, respectively, versus 41.0 cm3 for the classic type (P<0.001)). All cases were predominantly T2-weighted isointense and T1-weighted hypointense, and mean overall apparent diffusion coefficient values ranged from 1.05-1.10×10-3 mm2/s.
This retrospective international collaborative study showed classic type congenital mesoblastic nephroma predominantly presented as a homogeneous T2-weighted isointense mass with a typical concentric ring sign, whereas the cellular type appeared more heterogeneous. Future studies may use identified MRI characteristic of congenital mesoblastic nephroma for validation and for exploring the discriminative non-invasive value of MRI, especially from Wilms tumor.
This study aims to identify diagnostic MRI characteristics of congenital mesoblastic nephroma in the largest series of patients to date.
In this retrospective multicenter study, five SIOP-RTSG national review radiologists identified 52 diagnostic MRIs of histologically proven congenital mesoblastic nephromas. MRI was performed following SIOP-RTSG protocols, while radiologists assessed their national cases using a validated case report form.
Patients (24/52 classic, 11/52 cellular, and 15/52 mixed type congenital mesoblastic nephroma, 2/52 unknown) had a median age of 1 month (range 1 day-3 months). Classic type congenital mesoblastic nephroma appeared homogeneous with a lack of hemorrhage, necrosis and/or cysts, showing a concentric ring sign in 14 (58.3%) patients. Cellular and mixed type congenital mesoblastic nephroma appeared more heterogeneous and were larger (311.6 and 174.2 cm3, respectively, versus 41.0 cm3 for the classic type (P<0.001)). All cases were predominantly T2-weighted isointense and T1-weighted hypointense, and mean overall apparent diffusion coefficient values ranged from 1.05-1.10×10-3 mm2/s.
This retrospective international collaborative study showed classic type congenital mesoblastic nephroma predominantly presented as a homogeneous T2-weighted isointense mass with a typical concentric ring sign, whereas the cellular type appeared more heterogeneous. Future studies may use identified MRI characteristic of congenital mesoblastic nephroma for validation and for exploring the discriminative non-invasive value of MRI, especially from Wilms tumor.
摘要：
背景：先天性中胚层肾瘤是新生儿最常见的实体肾肿瘤。因此,<3个月的患者被建议接受前期肾切除术，而国际儿科肿瘤学会-肾肿瘤研究组(SIOP-RTSG)不鼓励≥3月龄患者在诊断时进行侵入性操作.然而，辨别先天性中胚层肾瘤，尤其是更常见的Wilms肿瘤，仅基于成像仍然很困难。最近,磁共振成像（MRI）已成为首选模式。针对先天性中胚层肾瘤的MRI特征的研究有限。
目的：本研究旨在确定迄今为止最大系列患者中先天性中胚层肾瘤的MRI诊断特征。
方法：在这项回顾性多中心研究中，5名SIOP-RTSG国家审查放射科医师鉴定出52例经组织学证实的先天性中胚层性肾瘤的诊断性MRI.MRI按照SIOP-RTSG方案进行,而放射科医生使用经过验证的病例报告表评估他们的国家病例。
结果：患者（24/52经典，11/52蜂窝，15/52混合型先天性中胚层肾瘤，2/52未知）的中位年龄为1个月（范围为1天-3个月）。经典型先天性中胚层肾瘤出现均一，缺乏出血，坏死和/或囊肿，14例(58.3%)患者出现同心环征。细胞型和混合型先天性中胚层肾瘤出现更多异质性和更大（分别为311.6和174.2cm3，而经典类型为41.0cm3（P<0.001））。所有病例均以T2加权等信号和T1加权低信号为主，平均总表观扩散系数值范围为1.05-1.10×10-3mm2/s。
结论：这项回顾性国际合作研究显示，典型型先天性中胚层肾瘤主要表现为具有典型同心环征的同质T2加权等强度肿块，而细胞类型显得更加异质。未来的研究可能会使用已确定的先天性中胚层肾瘤的MRI特征来进行验证和探索MRI的辨别非侵入性价值。尤其是Wilms肿瘤.
目的：本研究旨在确定迄今为止最大系列患者中先天性中胚层肾瘤的MRI诊断特征。
方法：在这项回顾性多中心研究中，5名SIOP-RTSG国家审查放射科医师鉴定出52例经组织学证实的先天性中胚层性肾瘤的诊断性MRI.MRI按照SIOP-RTSG方案进行,而放射科医生使用经过验证的病例报告表评估他们的国家病例。
结果：患者（24/52经典，11/52蜂窝，15/52混合型先天性中胚层肾瘤，2/52未知）的中位年龄为1个月（范围为1天-3个月）。经典型先天性中胚层肾瘤出现均一，缺乏出血，坏死和/或囊肿，14例(58.3%)患者出现同心环征。细胞型和混合型先天性中胚层肾瘤出现更多异质性和更大（分别为311.6和174.2cm3，而经典类型为41.0cm3（P<0.001））。所有病例均以T2加权等信号和T1加权低信号为主，平均总表观扩散系数值范围为1.05-1.10×10-3mm2/s。
结论：这项回顾性国际合作研究显示，典型型先天性中胚层肾瘤主要表现为具有典型同心环征的同质T2加权等强度肿块，而细胞类型显得更加异质。未来的研究可能会使用已确定的先天性中胚层肾瘤的MRI特征来进行验证和探索MRI的辨别非侵入性价值。尤其是Wilms肿瘤.
