Clinical and prognostic analysis of 42 children with malignant rhabdoid tumor of the kidney: a 7-year retrospective multi-center study

作者全名："Zhanghuang, Chenghao; Zhang, Zhaoxia; Zeng, Li; Yan, Bing; Tang, Haoyu; Wang, Jinkui; Liu, Xing; Wei, Guanghui; He, Dawei"

作者地址："[Zhanghuang, Chenghao; Zhang, Zhaoxia; Wang, Jinkui; Liu, Xing; Wei, Guanghui; He, Dawei] Chongqing Med Univ, Childrens Hosp,Minist Educ,Key Lab Child Dev & Di, China Int Sci & Technol Cooperat Base Child Dev &, Natl Clin Res Ctr Child Hlth & Disorders,Chongqin, Chongqing 400013, Peoples R China; [Zhanghuang, Chenghao; Yan, Bing; Tang, Haoyu] Kunming Med Univ, Childrens Hosp,Yunnan Prov Key Res Lab Pediat Maj, Kunming Childrens Hosp,Dept Urol, Yunnan Prov Clin Res Ctr Childrens Hlth & Dis, Kunming, Yunnan, Peoples R China; [Zeng, Li] Sichuan Univ, Dept Pediat Surg, West China Hosp, Chengdu, Peoples R China"

通信作者："He, DW (通讯作者)，Chongqing Med Univ, Childrens Hosp,Minist Educ,Key Lab Child Dev & Di, China Int Sci & Technol Cooperat Base Child Dev &, Natl Clin Res Ctr Child Hlth & Disorders,Chongqin, Chongqing 400013, Peoples R China."

来源：BMC PEDIATRICS

ESI学科分类：CLINICAL MEDICINE

WOS号：WOS:000867657400004

JCR分区：Q2

影响因子：2.4

年份：2022

卷号：22

期号：1

开始页：　

结束页：　

文献类型：Article

关键词：Children; Malignant rhabdoid tumor of the kidney; Multi-centers; Prognostic analysis; Diagnosis and treatment

摘要："Objective To discuss the clinical and prognostic indicators of pediatric malignant rhabdoid tumor of the kidney (MRTK), and to increase the understanding of the occurrence and development of MRTK. Methods From July 2014 to September 2021, all cases were confirmed by postoperative pathological examination. Among the 42 patients, there were 25 males and 17 females, with a median age of 10 (1-84) months. Abdominal mass or hematuria were the main clinical manifestations. Preoperative chemotherapy was performed in 9 cases (VC). The tumor stages were stage I-IV. Preoperative metastasis was found in 9 cases; the most common site was the lung. Postoperative patients received conventional chemotherapy, including VDACE regimen and UH-1 regimen. Among the 42 children in this group, survival at follow-up in this study was 26.2%(11/42). Results Preoperative anemia was found by univariate analysis, hypertension and hypercalcemia had shorter survival time. In addition, tumor-related factors had a significant impact on survival, with incomplete tumor resection, lymph node metastasis, stage III-IV had a lower survival rate. The impact of postoperative factors on survival included postoperative complications had a lower survival rate. The children were younger than 12 months, preoperative metastasis, no chemotherapy was performed after surgery was an independent risk factor for the prognosis of MRTK. Conclusion The main clinical manifestations about MRTK were abdominal mass and hematuria. Preoperative chemotherapy did not significantly improve the prognosis. Postoperative chemotherapy can significantly improve the survival rate. Diagnosis depends on clinical manifestations, imaging, histopathology, immunohistochemistry and other comprehensive judgment. Age less than 12 months, preoperative metastasis, and no postoperative chemotherapy were independent risk factors for prognosis."

基金机构：Chongqing Talent Program"lump sum project" [cstc2021ycjh-bgzxm0253]; Special Key Project of Chongqing Technology Innovation and Application Development [Cstc2019jscx-tjsbX0003]; Yunnan Education Department of Science Research Fund [2020J0228]; Kunming City Health Science and Technology Talent "1000" training Project [2020SW (Reserve)-112]; Kunming Health and Health Commission Health Research Project [2020-0201-001]; Kunming Medical Joint Project of Yunnan Science and Technology Department [202001AY070001-271]; Open research fund of Children's Health and Disease Clinical Medical Research Center of Yunnan Province [2022-ETYY-YJ-03]

基金资助正文："This study was supported by Chongqing Talent Program""lump sum project"" (No. cstc2021ycjh-bgzxm0253),Special Key Project of Chongqing Technology Innovation and Application Development(No. Cstc2019jscx-tjsbX0003),Yunnan Education Department of Science Research Fund (No. 2020J0228), Kunming City Health Science and Technology Talent ""1000"" training Project (No. 2020SW (Reserve)-112), Kunming Health and Health Commission Health Research Project (No. 2020-0201-001), Kunming Medical Joint Project of Yunnan Science and Technology Department (No. 202001AY070001-271), and Open research fund of Children's Health and Disease Clinical Medical Research Center of Yunnan Province(No.2022-ETYY-YJ-03).The funding bodies played no role in the study's design and collection, analysis and interpretation of data, and writing the manuscript."