和威尔姆斯相比,儿童非Wilms肾肿瘤由于其罕见的发生限制了典型成像模式的精确定义,因此人们对其了解较少。
这项研究旨在确定独特的影像学发现,儿童非Wilms肾肿瘤的人口学特征和预后。
从2007年1月至2018年12月,从我们的放射学档案中获得了207例诊断为原发性肾瘤变的患者。其中171人被诊断患有Wilms肿瘤,4例血管平滑肌脂肪瘤和1例肾性休息。其余31例诊断为原发性肾瘤变的患者被纳入这项回顾性研究。成像数据,年龄,性别,记录了预后和随访结果.
8名患者患有肾细胞癌,七个人患有透明细胞肉瘤,六个人患有中胚层肾瘤,四个有横纹肌样肿瘤,三人患有促纤维化小圆细胞瘤,其中2人患有囊性肾瘤,1人患有后肾间质瘤。诊断年龄>8岁为肾细胞癌和促纤维增生性小圆细胞瘤,横纹肌样瘤<5年,中胚层肾瘤<7个月。对任何肿瘤类型均无性别偏好。横纹肌样瘤的预后极差,因为我们研究所随访的所有患者均死亡,而在其他肿瘤中未发现复发。易位型肾细胞癌的T2加权信号强度较低,中胚层肾瘤主要是囊性肿块,透明细胞肉瘤通常在出现时较大,在纤维增生性小圆细胞肿瘤中可见广泛的无定形钙化。
对于小儿非Wilms肾肿瘤的鉴别诊断,年龄是最重要的因素,其次是转移/侵袭行为的倾向。了解这些肿瘤的特定影像学发现可能有助于缩小鉴别诊断范围。
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.