Department of Urology, National Children's Medical Center, Beijing Children's Hospital of Capital Medical University, No. 56 Nanlishi St, Xicheng District, Beijing, 100045, China.
BMC Urol. 2022 Jun 22;22(1):89. doi: 10.1186/s12894-022-01042-3.
Pediatric non-Wilms renal tumors (NWRTs), which comprise a small proportion of renal tumors, are a heterogeneous group of neoplasms with variable malignant potential, mortality, and response to treatment. We performed this study to determine the clinical characteristics, management and prognosis of children with Pediatric NWRTs.
Medical records of all patients (n = 139) treated for NWRTs over a 12-year period (2008.01-2019.10) at a single center were reviewed retrospectively.
The histopathological groups of NWRTs included malignant rhabdoid tumor of the kidney (MRTK) (n: 30, 21.6%), renal cell cancer (RCC) (n: 26,18.7%), clear cell sarcoma of the kidney (CCSK) (n: 24,17.3%), congenital mesoblastic nephroma (CMN) (n: 21,15.1%), cystic nephroma (CN) (n: 16,11.5%), metanephric tumors (n: 12, 8.6%), renal angiomyoliporma (RAML) (n: 3, 2.2%), renal primitive neuroectodermal tumor (n: 2, 1.4%), renal hemangioma (n: 2, 1.4%), inflammatory myofibroblastic tumor (n: 2, 1.4%), ossifying renal tumor of infancy (ORTI) (n: 1, 0.7%). The distribution of all malignant NWRTs, including MRTK, CCSK, RCC and PNET, according to stage was as follows: stages I (n = 26), II (n = 16), III (n = 29), and IV (n = 11). The summary table shows the treatment offered to children with NWRTs. A total of 123 children were followed up for an average of 42 months. Sixteen children were lost to follow-up. Tumor-free survival was observed in 94 children. One patient who suffered from RCC is currently receiving targeted therapy and survives with the tumor. Twenty-eight children (22.8%) died.
Pediatric NWRTs comprise 19.1% of all renal tumors in our single center. Most NWRTs can readily be distinguished using a range of immunohistochemical markers. Molecular genetic profiling has allowed much progress in the understanding of this group of tumors, making diagnosis and classification less difficult. The mainstay treatment of malignant NWRTs, including MRTK, CCSK, RCC and PNET, is comprehensive treatment. The mainstay treatment of benign NWRTs, including RAML, CN, ORTI, CMN, metanephric tumors, and renal hemangioma, is surgical resection alone and when the tumor diameter is smaller than 7 cm and the tumor locates in one pole, NSS can be performed.
小儿非 Wilms 肾肿瘤（NWRT）占肾脏肿瘤的一小部分，是一组具有不同恶性潜能、死亡率和治疗反应的异质性肿瘤。我们进行这项研究是为了确定儿童 NWRT 的临床特征、治疗方法和预后。
回顾性分析了 2008 年 1 月至 2019 年 10 月在一家单中心接受 NWRT 治疗的所有患者（n=139）的病历。
NWRT 的组织病理学组包括肾横纹肌样瘤（MRTK）（n=30，21.6%）、肾细胞癌（RCC）（n=26，18.7%）、肾透明细胞肉瘤（CCSK）（n=24，17.3%）、先天性中胚层肾瘤（CMN）（n=21，15.1%）、囊性肾瘤（CN）（n=16，11.5%）、中肾瘤（n=12，8.6%）、肾血管平滑肌脂肪瘤（RAML）（n=3，2.2%）、肾原始神经外胚层肿瘤（n=2，1.4%）、肾血管瘤（n=2，1.4%）、炎性肌纤维母细胞瘤（n=2，1.4%）、婴儿骨化性肾肿瘤（ORTI）（n=1，0.7%）。根据分期，所有恶性 NWRT 包括 MRTK、CCSK、RCC 和 PNET 的分布如下：I 期（n=26）、II 期（n=16）、III 期（n=29）和 IV 期（n=11）。总结表显示了 NWRT 患儿的治疗方法。123 例患儿平均随访 42 个月，16 例患儿失访。94 例患儿无肿瘤生存。1 例 RCC 患者目前正在接受靶向治疗，肿瘤存活。28 例（22.8%）患儿死亡。
小儿 NWRT 占我院单中心所有肾肿瘤的 19.1%。大多数 NWRT 可以通过一系列免疫组织化学标志物很容易地鉴别出来。分子遗传学分析在理解这组肿瘤方面取得了很大进展，使诊断和分类变得不那么困难。MRTK、CCSK、RCC 和 PNET 等恶性 NWRT 的主要治疗方法是综合治疗。RAML、CN、ORTI、CMN、中肾瘤和肾血管瘤等良性 NWRT 的主要治疗方法是单纯手术切除，当肿瘤直径小于 7cm 且位于一极时，可采用 NSS。
