Division of Pediatric Radiology, Department of Radiology, Hacettepe University School of Medicine, Sıhhiye, 06230, Ankara, Turkey.
Department of Radiology, Diyarbakır Children's Hospital, Diyarbakır, Turkey.
Pediatr Radiol. 2022 Dec;52(13):2557-2567. doi: 10.1007/s00247-022-05422-7. Epub 2022 Jun 28.
Compared to Wilms, non-Wilms renal tumors in children are less well understood due to their rare occurrence which limits precise definition of the typical imaging patterns.
This study aims to identify distinctive imaging findings, demographic characteristics and prognosis of pediatric non-Wilms renal tumors.
From January 2007 to December 2018, 207 patients with a diagnosis of primary kidney neoplasia were yielded from our radiology archive, 171 of whom were diagnosed with Wilms tumor, 4 with angiomyolipoma and one with nephrogenic rest. The remaining 31 patients with a diagnosis of primary kidney neoplasia were enrolled in this retrospective study. Imaging data, age, gender, prognosis and findings regarding follow-up were noted.
Eight patients had renal cell carcinoma, seven had clear cell sarcoma, six had mesoblastic nephroma, four had rhabdoid tumor, three had desmoplastic small round cell tumor, two had cystic nephroma and one had metanephric stromal tumor. The age of diagnosis was > 8 years for renal cell carcinoma and desmoplastic small round cell tumor, < 5 years for rhabdoid tumor and < 7 months for mesoblastic nephroma. There was no gender preference for any tumor type. The prognosis for rhabdoid tumor was extremely poor in that all the patients followed up in our institute were deceased, whereas no recurrence was found in other tumors. Translocation type renal cell carcinoma had lower T2-weighted signal intensity, mesoblastic nephroma was a predominantly cystic mass, clear cell sarcoma was generally larger at presentation and extensive amorphous calcifications were seen in desmoplastic small round cell tumor.
For the differential diagnosis of pediatric non-Wilms renal tumors, age is the most important factor, followed by propensity to metastasize/aggressive behavior of the mass. Knowledge of specific imaging findings of these tumors may help to narrow the differential diagnosis.
与肾母细胞瘤相比，儿童非肾母细胞瘤性肾肿瘤由于其罕见发生，限制了典型影像学表现的精确定义，因此了解较少。
本研究旨在确定小儿非肾母细胞瘤性肾肿瘤的独特影像学表现、人口统计学特征和预后。
从 2007 年 1 月至 2018 年 12 月，我们的放射学档案中共有 207 例原发性肾肿瘤患者，其中 171 例诊断为肾母细胞瘤，4 例诊断为血管平滑肌脂肪瘤，1 例诊断为肾生殖细胞瘤。其余 31 例原发性肾肿瘤患者被纳入本回顾性研究。记录了影像学数据、年龄、性别、预后和随访结果。
8 例患者为肾细胞癌，7 例为透明细胞肉瘤，6 例为中胚层肾瘤，4 例为横纹肌肉瘤，3 例为促纤维增生性小圆细胞肿瘤，2 例为囊性肾瘤，1 例为后肾间质瘤。肾细胞癌和促纤维增生性小圆细胞肿瘤的诊断年龄均>8 岁，横纹肌肉瘤的诊断年龄<5 岁，中胚层肾瘤的诊断年龄<7 个月。任何肿瘤类型均无性别偏好。横纹肌肉瘤的预后极差，我院随访的所有患者均死亡，而其他肿瘤均未发现复发。易位型肾细胞癌的 T2 加权信号强度较低，中胚层肾瘤主要为囊性肿块，透明细胞肉瘤一般较大，促纤维增生性小圆细胞肿瘤可见广泛的不定形钙化。
对于小儿非肾母细胞瘤性肾肿瘤的鉴别诊断，年龄是最重要的因素，其次是肿瘤的转移/侵袭性。了解这些肿瘤的特定影像学表现有助于缩小鉴别诊断范围。
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