{"title":"威尔姆斯肿瘤-审计。","authors":"Yogesh Kumar Sarin, Pute U Losu, Anita Nangia","doi":"10.4103/jiaps.jiaps_87_24","DOIUrl":null,"url":null,"abstract":"<p><strong>Background and aims: </strong>Outcome analysis of patients with Wilms' tumors (WT) is presented.</p><p><strong>Materials and methods: </strong>A retrospective analysis of 23 children having WT managed by a single surgeon over 3 years (2021-2024) using the International Society of Paediatric Oncology Umbrella protocol was done.</p><p><strong>Results: </strong>The median age at presentation was 36 months; 32 months and 24 months for the unilateral WT (uWT) (<i>n</i> = 19) and bilateral WT (bWT) (<i>n</i> = 4), respectively. M: F ratio was 2.3: 1. WTs were localized in 19 (uWT-16; bWT-3) and metastatic in 4 (uWT-3; bWT-1) patients. Core-needle biopsy was done in 22 patients (26 renal units). Pre-therapy contrast-enhanced computed tomography volumetry (<i>n</i> = 20) showed a median tumor volume of 1023 ml (range: 47-2680 ml). Post-neoadjuvant chemotherapy (NACT) median tumor burden (<i>n</i> = 19) was 612 ml (range 59-3775 ml). Post-NACT, tumor volume decreased in 11/18 patients but increased in seven patients. NACT was avoided in one neonate. Nephroureterectomy (including one with excision of bladder cuff) and nephron-sparing surgery were done in 17 and 10 renal units including 3 with multifocal WT, respectively. Risk stratification was intermediate in 21 and High in 2. Overall staging in 19 uWT included Stage I-7, Stage II-5, Stage III-4, and Stage IV-3 (local staging-stage I in 1 and stage II in 2). Local staging in 8 renal units with bWT was Stage I in 7 and II in 1. One stage IV uWT had bilateral pulmonary metastatectomy. Adjuvant chemotherapy has been completed in 18 patients; two patients are still on adjuvant chemotherapy; flank radiation was administered in six patients. Three patients with synchronous bWT died; two due to acute kidney injury in the immediate postoperative period and one with metastatic disease who had abandoned adjuvant chemotherapy after the 1<sup>st</sup> cycle. Another patient died of a huge metachronous tumor in the contralateral kidney after a year of completion of therapy. One patient had successful multimodality treatment of local relapse with liver metastasis. 1-year overall and event-free survivals are 84% and 76%, respectively.</p><p><strong>Conclusions: </strong>Excellent short-term results for localized uWT from a center in a low-middle-income country are reported.</p>","PeriodicalId":16069,"journal":{"name":"Journal of Indian Association of Pediatric Surgeons","volume":"29 6","pages":"600-607"},"PeriodicalIF":0.0000,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11649054/pdf/","citationCount":"0","resultStr":"{\"title\":\"Wilms' Tumor - An Audit.\",\"authors\":\"Yogesh Kumar Sarin, Pute U Losu, Anita Nangia\",\"doi\":\"10.4103/jiaps.jiaps_87_24\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Background and aims: </strong>Outcome analysis of patients with Wilms' tumors (WT) is presented.</p><p><strong>Materials and methods: </strong>A retrospective analysis of 23 children having WT managed by a single surgeon over 3 years (2021-2024) using the International Society of Paediatric Oncology Umbrella protocol was done.</p><p><strong>Results: </strong>The median age at presentation was 36 months; 32 months and 24 months for the unilateral WT (uWT) (<i>n</i> = 19) and bilateral WT (bWT) (<i>n</i> = 4), respectively. M: F ratio was 2.3: 1. WTs were localized in 19 (uWT-16; bWT-3) and metastatic in 4 (uWT-3; bWT-1) patients. Core-needle biopsy was done in 22 patients (26 renal units). Pre-therapy contrast-enhanced computed tomography volumetry (<i>n</i> = 20) showed a median tumor volume of 1023 ml (range: 47-2680 ml). Post-neoadjuvant chemotherapy (NACT) median tumor burden (<i>n</i> = 19) was 612 ml (range 59-3775 ml). Post-NACT, tumor volume decreased in 11/18 patients but increased in seven patients. NACT was avoided in one neonate. Nephroureterectomy (including one with excision of bladder cuff) and nephron-sparing surgery were done in 17 and 10 renal units including 3 with multifocal WT, respectively. Risk stratification was intermediate in 21 and High in 2. Overall staging in 19 uWT included Stage I-7, Stage II-5, Stage III-4, and Stage IV-3 (local staging-stage I in 1 and stage II in 2). Local staging in 8 renal units with bWT was Stage I in 7 and II in 1. One stage IV uWT had bilateral pulmonary metastatectomy. Adjuvant chemotherapy has been completed in 18 patients; two patients are still on adjuvant chemotherapy; flank radiation was administered in six patients. Three patients with synchronous bWT died; two due to acute kidney injury in the immediate postoperative period and one with metastatic disease who had abandoned adjuvant chemotherapy after the 1<sup>st</sup> cycle. Another patient died of a huge metachronous tumor in the contralateral kidney after a year of completion of therapy. One patient had successful multimodality treatment of local relapse with liver metastasis. 1-year overall and event-free survivals are 84% and 76%, respectively.</p><p><strong>Conclusions: </strong>Excellent short-term results for localized uWT from a center in a low-middle-income country are reported.</p>\",\"PeriodicalId\":16069,\"journal\":{\"name\":\"Journal of Indian Association of Pediatric Surgeons\",\"volume\":\"29 6\",\"pages\":\"600-607\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2024-11-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11649054/pdf/\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Journal of Indian Association of Pediatric Surgeons\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.4103/jiaps.jiaps_87_24\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2024/11/5 0:00:00\",\"PubModel\":\"Epub\",\"JCR\":\"Q3\",\"JCRName\":\"Medicine\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Journal of Indian Association of Pediatric Surgeons","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.4103/jiaps.jiaps_87_24","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2024/11/5 0:00:00","PubModel":"Epub","JCR":"Q3","JCRName":"Medicine","Score":null,"Total":0}
Background and aims: Outcome analysis of patients with Wilms' tumors (WT) is presented.
Materials and methods: A retrospective analysis of 23 children having WT managed by a single surgeon over 3 years (2021-2024) using the International Society of Paediatric Oncology Umbrella protocol was done.
Results: The median age at presentation was 36 months; 32 months and 24 months for the unilateral WT (uWT) (n = 19) and bilateral WT (bWT) (n = 4), respectively. M: F ratio was 2.3: 1. WTs were localized in 19 (uWT-16; bWT-3) and metastatic in 4 (uWT-3; bWT-1) patients. Core-needle biopsy was done in 22 patients (26 renal units). Pre-therapy contrast-enhanced computed tomography volumetry (n = 20) showed a median tumor volume of 1023 ml (range: 47-2680 ml). Post-neoadjuvant chemotherapy (NACT) median tumor burden (n = 19) was 612 ml (range 59-3775 ml). Post-NACT, tumor volume decreased in 11/18 patients but increased in seven patients. NACT was avoided in one neonate. Nephroureterectomy (including one with excision of bladder cuff) and nephron-sparing surgery were done in 17 and 10 renal units including 3 with multifocal WT, respectively. Risk stratification was intermediate in 21 and High in 2. Overall staging in 19 uWT included Stage I-7, Stage II-5, Stage III-4, and Stage IV-3 (local staging-stage I in 1 and stage II in 2). Local staging in 8 renal units with bWT was Stage I in 7 and II in 1. One stage IV uWT had bilateral pulmonary metastatectomy. Adjuvant chemotherapy has been completed in 18 patients; two patients are still on adjuvant chemotherapy; flank radiation was administered in six patients. Three patients with synchronous bWT died; two due to acute kidney injury in the immediate postoperative period and one with metastatic disease who had abandoned adjuvant chemotherapy after the 1st cycle. Another patient died of a huge metachronous tumor in the contralateral kidney after a year of completion of therapy. One patient had successful multimodality treatment of local relapse with liver metastasis. 1-year overall and event-free survivals are 84% and 76%, respectively.
Conclusions: Excellent short-term results for localized uWT from a center in a low-middle-income country are reported.
期刊介绍：
Journal of Indian Association of Pediatric Surgeons is the official organ of Indian Association of Pediatric Surgeons. The journal started its journey in October 1995 under the Editor-in-Chief Prof. Subir K Chatterjee. An advisory board was formed with well-versed internationally reputed senior members of our society like Late Prof. R K Gandhi, Prof. I C Pathak, Prof. P Upadhyay, Prof. T Dorairajan and many more. since then the journal is published quarterly uninterrupted. The journal publishes original articles, case reports, review articles and technical innovations. Special issues on different subjects are published every year. There have been several contributions from overseas experts.
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