66 research outputs found
ASO visual abstract: the value of the sentinel node procedure in pediatric extremity Rhabdomyosarcoma: a systematic review and retrospective cohort study
Development and application of statistical models for medical scientific researc
Value of the sentinel node procedure in pediatric extremity rhabdomyosarcoma: a systematic review and retrospective cohort study
Background Our aim is to show whether the sentinel node procedure (SNP) is recommendable for pediatric patients with extremity rhabdomyosarcoma (RMS). Lymph node metastases are an important prognostic factor in pediatric patients with extremity RMS. Accurate nodal staging is necessary to treat the patient accordingly. An alternative to the current recommended lymph node sampling is the sentinel node procedure (SNP). Methods A systematic review was performed summarizing all published cases of SNP in addition to 13 cases from our hospital and 8 cases from two other hospitals that have not been published before. Results For all patients (n = 55), at least one SLN was identified, but the SNP technique used was not uniform. The SNP changed the nodal classification of eight patients (17.0%) and had a false-negative rate of 10.5%. Conclusions The SNP is recommendable for pediatric patients with extremity RMS. It can change lymph node status and can be used to sample patients in a more targeted way than nodal sampling alone. Therefore, we recommend use of the SNP in addition to clinical and radiological nodal assessment for pediatric patients with extremity RMS.Development and application of statistical models for medical scientific researc
Congenital mesoblastic nephroma 50 years after its recognition: A narrative review
Congenital mesoblastic nephroma (CMN) is a rare pediatric renal tumor with low malignant potential that most commonly occurs early in infancy. Treatment strategies are based on the few published CMN series, while a significant number of CMN patients have been described in case reports. The aim of this narrative review was to create an upâtoâdate overview of the literature. Complete surgical removal is curative in most cases. The risk of treatmentârelated mortality (both surgeryâ and chemotherapyârelated) is relatively high in the first weeks of life, indicating that these young patients deserve special attention with respect to timing and type of treatment
Characteristics and outcome of pediatric renal cell carcinoma patients registered in the International Society of Pediatric Oncology (SIOP) 93â01, 2001 and UKâIMPORT database: A report of the SIOPâRenal Tumor Study Group
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 OncologyâRenal Tumor Study Group (SIOPâRTSG). Pediatric patients with histologically confirmed RCC, registered in SIOP 93â01, 2001 and UKâIMPORT databases, were included. Eventâfree survival (EFS) and overall survival (OS) were analyzed using the KaplanâMeier 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 (MiTâRCC) (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 TFEâcytogenetics 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 5âyear (5y) EFS and 5yâOS of the total group was 70.5% (95% CI = 61.7%â80.6%) and 84.5% (95% CI = 77.5%â92.2%), respectively. Estimated 5yâOS 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 TFEâtesting
Prognostic significance of age in 5631 patients with Wilms tumour prospectively registered in International Society of Paediatric Oncology (SIOP) 93-01 and 2001
Background
To enhance risk stratification for Wilms tumour (WT) in a pre-operative chemotherapy setting, we explored the prognostic significance and optimal age cutoffs in patients treated according to International Society of Paediatric Oncology Renal Tumour Study Group (SIOP-RTSG) protocols.
Methods
Patients(6 months-18 years) with unilateral WT were selected from prospective SIOP 93â01 and 2001 studies(1993â2016). Martingale residual analysis was used to explore optimal age cutoffs. Outcome according to age was analyzed by uni- and multivariable analysis, adjusted for sex, biopsy(yes/no), stage, histology and tumour volume at surgery.
Results
5631 patients were included; median age was 3.4 years(IQR: 2â5.1). Estimated 5-year event-free survival (EFS) and overall survival (OS) were 85%(95%CI 83.5â85.5) and 93%(95%CI 92.0â93.4). Martingale residual plots detected no optimal age cutoffs. Multivariable analysis showed lower EFS with increasing age(linear trend P<0.001). Using previously described age categories, EFS was lower for patients aged 2-4(HR 1.34, P = 0.02), 4-10(HR 1.83, P<0.0001) and 10â18 years(HR 1.74, P = 0.01) as compared to patients aged 6 months-2 years. OS was lower for patients 4â10 years(HR 1.67, P = 0.01) and 10â18 years(HR 1.87, P = 0.04), but not for 2â4 years(HR 1.29, P = 0.23). Higher stage, histological risk group and tumour volume were independent adverse prognostic factors.
Conclusion
Although optimal age cutoffs could not be identified, we demonstrated the prognostic significance of age as well as previously described cutoffs for EFS (2 and 4 years) and OS (4 years) in children with WT treated with pre-operative chemotherapy. These findings encourage the consideration of age in the design of future SIOP-RTSG protocols
Rationale for the treatment of children with CCSK in the UMBRELLA SIOP-RTSG 2016 protocol
The International Society of Paediatric Oncology-Renal Tumour Study Group (SIOP-RTSG) has developed a new protocol for the diagnosis, treatment, and follow-up monitoring of childhood renal tumours-the UMBRELLA SIOP-RTSG 2016 protocol (the UMBRELLA protocol). This protocol has been designed to continue international collaboration in the treatment of childhood renal tumours and will be implemented in over 50 different countries. Clear cell sarcoma of the kidney, which is a rare paediatric renal tumour that most commonly occurs in childre
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