背景:小儿非Wilms肾肿瘤(NWRTs),其中包括一小部分肾脏肿瘤,是一组具有可变恶性潜能的异质性肿瘤,死亡率,以及对治疗的反应。我们进行这项研究以确定临床特征,儿童NWRT的治疗和预后。
方法:回顾性分析了在一个中心接受12年(2008.01-2019.10)NWRTs治疗的所有患者(n=139)的病历。
结果:NWRT的组织病理学组包括肾脏恶性横纹肌样瘤(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%),后肾肿瘤(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%),婴儿期骨化肾肿瘤(ORTI)(n:1,0.7%)。所有恶性NWRT的分布,包括MRTK,CCSK,RCC和PNET,根据阶段如下:阶段I(n=26),II(n=16),III(n=29),和IV(n=11)。汇总表显示了为患有NWRT的儿童提供的治疗。对123例患儿进行平均42个月的随访。16名儿童失去了后续行动。在94名儿童中观察到无瘤生存。一名患有RCC的患者目前正在接受靶向治疗并与肿瘤一起存活。28名儿童(22.8%)死亡。
结论:小儿NWRT占我们单中心所有肾肿瘤的19.1%。大多数NWRT可以使用一系列免疫组织化学标记容易地区分。分子遗传学分析在理解这类肿瘤方面取得了很大进展,使诊断和分类变得不那么困难。恶性NWRT的主要治疗方法,包括MRTK,CCSK,RCC和PNET,是综合治疗。良性NWRT的主要治疗方法,包括RAML,CN,ORTI,CMN,后肾肿瘤,肾血管瘤,是单独的手术切除,当肿瘤直径小于7厘米,肿瘤位于一个极点时,可以执行NSS。
BACKGROUND: 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.
METHODS: 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.
RESULTS: 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.
CONCLUSIONS: 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.