Children's Hospital of Philadelphia, Philadelphia, PA, USA.
Pediatr Nephrol. 2021 Jul;36(7):1775-1784. doi: 10.1007/s00467-020-04835-6. Epub 2021 Feb 13.
Patients with tuberous sclerosis complex (TSC) can develop solid kidney masses from childhood. Imaging surveillance is done to detect renal cell carcinoma (RCC) and angiomyolipomas (AML), including AMLs at risk for hemorrhage. Intravenous contrast-enhanced ultrasound (CEUS) may be useful for screening as ultrasound is well tolerated by children and ultrasound contrast agents (UCA) are not nephrotoxic.
Retrospective review of kidney CEUS exams of pediatric TSC patients. Qualitative CEUS analysis by consensus of 3 radiologists assessed rate, intensity, and pattern of lesion enhancement. Quantitative CEUS analysis was performed using Vuebox®. Where available, abdominal MRI was analyzed qualitatively for the same features and quantitatively by in-house-developed software. Time-intensity curves were generated from both CEUS and MRI where possible. Appearance of lesions were compared between CEUS and MRI and histology where available.
Nine masses in 5 patients included one histologically proven RCC and 8 AMLs diagnosed by imaging. Quantitative CEUS of RCC showed malignant features including increased peak enhancement 162%, rapid wash-in rate 162%, and elevated washout rate 156% compared to normal kidney tissue; versus AML which was 68%, 105%, and 125%, respectively. All masses were hypoenhancing on MRI compared to normal kidney tissue; MR dynamic contrast study offered no distinction between RCC and AML. The only MRI feature differentiating RCC from AML was absence of fat.
Temporal resolution afforded by CEUS was useful to distinguish malignant from benign kidney masses. CEUS may prove useful for screening, characterizing, and follow-up of kidney lesions in pediatric TSC patients.
患有结节性硬化症(TSC)的患者在儿童时期可能会出现肾脏实性肿块。影像学监测用于检测肾细胞癌(RCC)和血管平滑肌脂肪瘤(AML),包括有出血风险的 AML。静脉内超声造影(CEUS)可能有助于筛查,因为超声对儿童的耐受性良好,且超声造影剂(UCA)无肾毒性。
回顾性分析儿科 TSC 患者的肾脏 CEUS 检查结果。由 3 名放射科医生共识进行定性 CEUS 分析,评估病变增强的速率、强度和模式。使用 Vuebox®进行定量 CEUS 分析。在有条件的情况下,对腹部 MRI 进行相同特征的定性分析,并使用内部开发的软件进行定量分析。尽可能从 CEUS 和 MRI 生成时间-强度曲线。比较 CEUS 和 MRI 以及有条件时的组织学上的病变外观。
5 例患者的 9 个肿块中包括 1 个经组织学证实的 RCC 和 8 个 AML。RCC 的定量 CEUS 显示恶性特征,包括增强峰值增加 162%、快速洗脱率 162%和洗脱率升高 156%,与正常肾组织相比;而 AML 分别为 68%、105%和 125%。与正常肾组织相比,所有肿块在 MRI 上均呈低增强;MR 动态对比研究未能区分 RCC 和 AML。唯一能区分 RCC 和 AML 的 MRI 特征是缺乏脂肪。
CEUS 提供的时间分辨率有助于区分恶性和良性肾脏肿块。CEUS 可能有助于儿科 TSC 患者的肾脏病变筛查、特征描述和随访。