Non-Wilms' renal tumors in children: experience with 139 cases treated at a single center
Abstract 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, manageme...
| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
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BMC
2022-06-01
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| Series: | BMC Urology |
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| Online Access: | https://doi.org/10.1186/s12894-022-01042-3 |
| _version_ | 1828773487964061696 |
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| author | Yi Wei Fang Hong Cheng Song Ning Sun Wei Ping Zhang |
| author_facet | Yi Wei Fang Hong Cheng Song Ning Sun Wei Ping Zhang |
| author_sort | Yi Wei Fang |
| collection | DOAJ |
| description | Abstract 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. |
| first_indexed | 2024-12-11T15:04:08Z |
| format | Article |
| id | doaj.art-57f0eeb750a24af282e10a3a50008b40 |
| institution | Directory Open Access Journal |
| issn | 1471-2490 |
| language | English |
| last_indexed | 2024-12-11T15:04:08Z |
| publishDate | 2022-06-01 |
| publisher | BMC |
| record_format | Article |
| series | BMC Urology |
| spelling | doaj.art-57f0eeb750a24af282e10a3a50008b402022-12-22T01:01:00ZengBMCBMC Urology1471-24902022-06-012211910.1186/s12894-022-01042-3Non-Wilms' renal tumors in children: experience with 139 cases treated at a single centerYi Wei Fang0Hong Cheng Song1Ning Sun2Wei Ping Zhang3Department of Urology, National Children’s Medical Center, Beijing Children’s Hospital of Capital Medical UniversityDepartment of Urology, National Children’s Medical Center, Beijing Children’s Hospital of Capital Medical UniversityDepartment of Urology, National Children’s Medical Center, Beijing Children’s Hospital of Capital Medical UniversityDepartment of Urology, National Children’s Medical Center, Beijing Children’s Hospital of Capital Medical UniversityAbstract 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.https://doi.org/10.1186/s12894-022-01042-3ChildrenNon-Wilms' renal tumorsManagement |
| spellingShingle | Yi Wei Fang Hong Cheng Song Ning Sun Wei Ping Zhang Non-Wilms' renal tumors in children: experience with 139 cases treated at a single center BMC Urology Children Non-Wilms' renal tumors Management |
| title | Non-Wilms' renal tumors in children: experience with 139 cases treated at a single center |
| title_full | Non-Wilms' renal tumors in children: experience with 139 cases treated at a single center |
| title_fullStr | Non-Wilms' renal tumors in children: experience with 139 cases treated at a single center |
| title_full_unstemmed | Non-Wilms' renal tumors in children: experience with 139 cases treated at a single center |
| title_short | Non-Wilms' renal tumors in children: experience with 139 cases treated at a single center |
| title_sort | non wilms renal tumors in children experience with 139 cases treated at a single center |
| topic | Children Non-Wilms' renal tumors Management |
| url | https://doi.org/10.1186/s12894-022-01042-3 |
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