Clinical and prognostic analysis of 42 children with malignant rhabdoid tumor of the kidney: a 7-year retrospective multi-center study
Abstract 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 pathologic...
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BMC
2022-10-01
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| Series: | BMC Pediatrics |
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| Online Access: | https://doi.org/10.1186/s12887-022-03643-1 |
| _version_ | 1827982772722991104 |
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| author | Chenghao Zhanghuang Zhaoxia Zhang Li Zeng Bing Yan Haoyu Tang Jinkui Wang Xing Liu Guanghui Wei Dawei He |
| author_facet | Chenghao Zhanghuang Zhaoxia Zhang Li Zeng Bing Yan Haoyu Tang Jinkui Wang Xing Liu Guanghui Wei Dawei He |
| author_sort | Chenghao Zhanghuang |
| collection | DOAJ |
| description | Abstract 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. |
| first_indexed | 2024-04-09T22:37:31Z |
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| id | doaj.art-c7b3ff8168684204900bde8a5f3d2d76 |
| institution | Directory Open Access Journal |
| issn | 1471-2431 |
| language | English |
| last_indexed | 2024-04-09T22:37:31Z |
| publishDate | 2022-10-01 |
| publisher | BMC |
| record_format | Article |
| series | BMC Pediatrics |
| spelling | doaj.art-c7b3ff8168684204900bde8a5f3d2d762023-03-22T12:23:39ZengBMCBMC Pediatrics1471-24312022-10-0122111210.1186/s12887-022-03643-1Clinical and prognostic analysis of 42 children with malignant rhabdoid tumor of the kidney: a 7-year retrospective multi-center studyChenghao Zhanghuang0Zhaoxia Zhang1Li Zeng2Bing Yan3Haoyu Tang4Jinkui Wang5Xing Liu6Guanghui Wei7Dawei He8Department of Urology, Chongqing Key Laboratory of Children Urogenital Development and Tissue Engineering, Chongqing Key Laboratory of Pediatrics, Ministry of Education Key Laboratory of Child Development and Disorders, National Clinical Research Center for Child Health and Disorders, China International Science and Technology Cooperation base of Child development and Critical Disorders, Children’s Hospital of Chongqing Medical UniversityDepartment of Urology, Chongqing Key Laboratory of Children Urogenital Development and Tissue Engineering, Chongqing Key Laboratory of Pediatrics, Ministry of Education Key Laboratory of Child Development and Disorders, National Clinical Research Center for Child Health and Disorders, China International Science and Technology Cooperation base of Child development and Critical Disorders, Children’s Hospital of Chongqing Medical UniversityDepartment of Pediatric Surgical, West China Hospital, Sichuan UniversityDepartment of Urology, Yunnan Provincial Key Research Laboratory of Pediatric Major Diseases, Yunnan Province Clinical Research Center for Children’s Health and Disease, Kunming Children’s Hospital, Children’s Hospital Affiliated to Kunming Medical UniversityDepartment of Urology, Yunnan Provincial Key Research Laboratory of Pediatric Major Diseases, Yunnan Province Clinical Research Center for Children’s Health and Disease, Kunming Children’s Hospital, Children’s Hospital Affiliated to Kunming Medical UniversityDepartment of Urology, Chongqing Key Laboratory of Children Urogenital Development and Tissue Engineering, Chongqing Key Laboratory of Pediatrics, Ministry of Education Key Laboratory of Child Development and Disorders, National Clinical Research Center for Child Health and Disorders, China International Science and Technology Cooperation base of Child development and Critical Disorders, Children’s Hospital of Chongqing Medical UniversityDepartment of Urology, Chongqing Key Laboratory of Children Urogenital Development and Tissue Engineering, Chongqing Key Laboratory of Pediatrics, Ministry of Education Key Laboratory of Child Development and Disorders, National Clinical Research Center for Child Health and Disorders, China International Science and Technology Cooperation base of Child development and Critical Disorders, Children’s Hospital of Chongqing Medical UniversityDepartment of Urology, Chongqing Key Laboratory of Children Urogenital Development and Tissue Engineering, Chongqing Key Laboratory of Pediatrics, Ministry of Education Key Laboratory of Child Development and Disorders, National Clinical Research Center for Child Health and Disorders, China International Science and Technology Cooperation base of Child development and Critical Disorders, Children’s Hospital of Chongqing Medical UniversityDepartment of Urology, Chongqing Key Laboratory of Children Urogenital Development and Tissue Engineering, Chongqing Key Laboratory of Pediatrics, Ministry of Education Key Laboratory of Child Development and Disorders, National Clinical Research Center for Child Health and Disorders, China International Science and Technology Cooperation base of Child development and Critical Disorders, Children’s Hospital of Chongqing Medical UniversityAbstract 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.https://doi.org/10.1186/s12887-022-03643-1ChildrenMalignant rhabdoid tumor of the kidneyMulti-centersPrognostic analysisDiagnosis and treatment |
| spellingShingle | Chenghao Zhanghuang Zhaoxia Zhang Li Zeng Bing Yan Haoyu Tang Jinkui Wang Xing Liu Guanghui Wei Dawei He Clinical and prognostic analysis of 42 children with malignant rhabdoid tumor of the kidney: a 7-year retrospective multi-center study BMC Pediatrics Children Malignant rhabdoid tumor of the kidney Multi-centers Prognostic analysis Diagnosis and treatment |
| title | Clinical and prognostic analysis of 42 children with malignant rhabdoid tumor of the kidney: a 7-year retrospective multi-center study |
| title_full | Clinical and prognostic analysis of 42 children with malignant rhabdoid tumor of the kidney: a 7-year retrospective multi-center study |
| title_fullStr | Clinical and prognostic analysis of 42 children with malignant rhabdoid tumor of the kidney: a 7-year retrospective multi-center study |
| title_full_unstemmed | Clinical and prognostic analysis of 42 children with malignant rhabdoid tumor of the kidney: a 7-year retrospective multi-center study |
| title_short | Clinical and prognostic analysis of 42 children with malignant rhabdoid tumor of the kidney: a 7-year retrospective multi-center study |
| title_sort | clinical and prognostic analysis of 42 children with malignant rhabdoid tumor of the kidney a 7 year retrospective multi center study |
| topic | Children Malignant rhabdoid tumor of the kidney Multi-centers Prognostic analysis Diagnosis and treatment |
| url | https://doi.org/10.1186/s12887-022-03643-1 |
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