ArticleInternational journal of cancer2021
Characteristics and outcome of pediatric renal cell carcinoma patients registered in the International Society of Pediatric Oncology (SIOP) 93-01, 2001 and UK-IMPORT database: A report of the SIOP-Renal Tumor Study Group.
Article in International journal of cancer, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 23 papers.
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Who cites it
23 citing papers in PubMed, 39 citations in OpenAlex.
- Relapsed rhabdoid tumours and other non-nephroblastoma childhood and adolescent kidney tumours: perspectives from the HARMONICA collaboration.Nature reviews. Urology · 2026Review
- Integrated Molecular, Genomic, and Clinical Characterization of Pediatric and Adolescent Translocation Renal Cell Carcinoma: A Report from the Children's Oncology Group.Biomedicines · 2026Article
- Non-Wilms Renal Tumours in Children: The Republic of Ireland Experience.Children (Basel, Switzerland) · 2026Article
- Improving the diagnosis of renal tumours of young people through integrated molecular analysis.Journal of cancer research and clinical oncology · 2026Article
- NMRK2 leads to the depletion of CD8Oncogene · 2025Article
- Paediatric Renal Tumors: A State-of-the-Art Review.Current oncology reports · 2025Review
- Management of translocation carcinomas of the kidney.Translational cancer research · 2024Review
- Pediatric thyroid-like follicular renal cell carcinoma-a post-neuroblastoma case with comprehensive genomic profiling data.Virchows Archiv : an international journal of pathology · 2024Article
- Pediatric with localized renal cell carcinoma (RCC).Radiology case reports · 2024Article
- Treatment of metastatic TFE3 microphthalmia transcription factor translocation renal cell carcinoma: a case report.Translational pediatrics · 2024Article
- Clinicopathologic Classification of Renal Cell Carcinoma in Patients ≤40 Years Old From Peru.International journal of surgical pathology · 2024Article
- Targeted therapies in children with renal cell carcinoma (RCC): An International Society of Pediatric Oncology-Renal Tumor Study Group (SIOP-RTSG)-related retrospective descriptive study.Cancer medicine · 2024Article
- CT-based identification of pediatric non-Wilms tumors using convolutional neural networks at a single center.Pediatric research · 2023Article
- PI3K/AKT/mTOR Dysregulation and Reprogramming Metabolic Pathways in Renal Cancer: Crosstalk with the VHL/HIF Axis.International journal of molecular sciences · 2023Review
- Article
- Review
- MiT/TFE Family Renal Cell Carcinoma.Genes · 2023Review
- Clear Cell Renal Cell Carcinoma, Diagnostic and Therapeutic Difficulties, Case Report and Literature Review.Medicina (Kaunas, Lithuania) · 2022Review
- The genomic landscape of pediatric renal cell carcinomas.iScience · 2022Article
- Epidemiological and Histopathological Characteristics of Renal Cell Carcinoma in Somalia.Cancer management and research · 2022Article
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29 authors at 20 institutions in 11 countries.
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Abstract
In children, renal cell carcinoma (RCC) is rare. This study is the first report of pediatric patients with RCC registered by the International Society of Pediatric Oncology-Renal Tumor Study Group (SIOP-RTSG). Pediatric patients with histologically confirmed RCC, registered in SIOP 93-01, 2001 and UK-IMPORT databases, were included. Event-free survival (EFS) and overall survival (OS) were analyzed using the Kaplan-Meier method. Between 1993 and 2019, 122 pediatric patients with RCC were registered. Available detailed data (n = 111) revealed 56 localized, 30 regionally advanced, 25 metastatic and no bilateral cases. Histological classification according to World Health Organization 2004, including immunohistochemical and molecular testing for transcription factor E3 (TFE3) and/or EB (TFEB) translocation, was available for 65/122 patients. In this group, the most common histological subtypes were translocation type RCC (MiT-RCC) (36/64, 56.3%), papillary type (19/64, 29.7%) and clear cell type (4/64, 6.3%). One histological subtype was not reported. In the remaining 57 patients, translocation testing could not be performed, or TFE-cytogenetics and/or immunohistochemistry results were missing. In this group, the most common RCC histological subtypes were papillary type (21/47, 44.7%) and clear cell type (11/47, 23.4%). Ten histological subtypes were not reported. Estimated 5-year (5y) EFS and 5y OS of the total group was 70.5% (95% CI = 61.7%-80.6%) and 84.5% (95% CI = 77.5%-92.2%), respectively. Estimated 5y OS for localized, regionally advanced, and metastatic disease was 96.8%, 92.3%, and 45.6%, respectively. In conclusion, the registered pediatric patients with RCC showed a reasonable outcome. Survival was substantially lower for patients with metastatic disease. This descriptive study stresses the importance of full, prospective registration including TFE-testing.
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