Department of Radiology and Nuclear Medicine, University Medical Center Utrecht/Wilhelmina Children's Hospital, Utrecht University, Heidelberglaan 100, 3584 CX, Utrecht, The Netherlands.
Princess Máxima Center for Pediatric Oncology, Utrecht, The Netherlands.
Pediatr Radiol. 2024 May;54(6):965-976. doi: 10.1007/s00247-024-05918-4. Epub 2024 Apr 13.
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 cm, respectively, versus 41.0 cm 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 mm/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.
先天性中胚层肾瘤是新生儿中最常见的肾脏实体瘤。因此,建议年龄<3 个月的患者行 upfront 肾切除术,而国际小儿肿瘤学会-肾脏肿瘤研究组(SIOP-RTSG)不建议年龄≥3 个月的患者在诊断时进行侵袭性操作。然而,仅凭影像学很难区分先天性中胚层肾瘤,尤其是更常见的肾母细胞瘤。最近,磁共振成像(MRI)已成为首选的检查方法。目前,关于先天性中胚层肾瘤 MRI 特征的研究有限。
本研究旨在确定迄今为止最大的患者系列中先天性中胚层肾瘤的诊断性 MRI 特征。
在这项回顾性多中心研究中,5 名 SIOP-RTSG 全国审查放射科医生从组织学证实的先天性中胚层肾瘤中确定了 52 例诊断性 MRI。MRI 是按照 SIOP-RTSG 方案进行的,而放射科医生使用经过验证的病例报告表对其国家病例进行了评估。
患者(24/52 例经典型、11/52 例细胞型和 15/52 例混合性先天性中胚层肾瘤、2/52 例未知)的中位年龄为 1 个月(范围为 1 天至 3 个月)。经典型先天性中胚层肾瘤表现为均匀一致,无出血、坏死和/或囊肿,14 例(58.3%)患者呈同心环征。细胞型和混合性先天性中胚层肾瘤表现为更不均匀,且更大(分别为 311.6 和 174.2 cm,而经典型为 41.0 cm(P<0.001))。所有病例在 T2 加权像上均呈等信号,T1 加权像上呈低信号,平均表观扩散系数值范围为 1.05-1.10×10 mm/s。
这项回顾性国际合作研究表明,经典型先天性中胚层肾瘤主要表现为均匀的 T2 加权等信号肿块,伴有典型的同心环征,而细胞型则表现为更不均匀。未来的研究可能会利用已确定的先天性中胚层肾瘤的 MRI 特征进行验证,并探索 MRI 的鉴别性无创价值,特别是与肾母细胞瘤的鉴别。