Advances and changes in the treatment of children with nephroblastoma

ArticleinAdvances in Clinical and Experimental Medicine 21(6):909-820 · November 2012with11 Reads
Source: PubMed
Wilms' tumor or nephroblastoma is the most common malignant tumor stemming from kidney cells and second only to neuroblastoma when it comes to extracranial solid tumors in children. The results of nephroblastoma treatment are a perfect example of therapeutic success resulting from an interdisciplinary approach to the problem and the cooperation of pediatric surgeons, pediatric oncologists, pathologists, radiologists and radiotherapists leading to precise diagnoses and the selection of the optimal treatment. At the end of the sixties, international research teams began studying the best treatment for this tumor in children. In Europe, it was the International Society of Paediatric Oncology (SIOP), which has used the working name SIOP- RTSG (Renal Tumor Study Group - Group for the Study of Kidney tumors) since 2008 and in North America NWTS (National Wilms' Tumor Study - The National Committee for Research on Wilms' tumor). Summarizing the experience and knowledge on the treatment of nephroblastoma, it should be noted that, despite years of research and information exchange, uniform guidelines have not yet been developed, and there are still differences in treatment of this tumor. The biggest differences are between the "American" treatment recommended by the NWTS and the "European" by SIOP. In the first it is recommended to start treatment from the surgical removal of the tumor, even in the case of disseminated disease with the presence of metastases in the lungs. The treatment method is chosen by the institution managing the patient; for this reason on the American continent in Brazil, Wilms' tumor is treated according to the recommendations of "European" protocols (SIOP) and some institutions in Europe, for example in Italy, treat patients with nephroblastoma according to the "American" protocols recommended by the NWTS; until recently, focal disease was treated with primary nephrectomy in the UK.
    • "Measuring only a mean or median could conceal relevant information . Therefore we also assessed the variability within and between observers for percentage of pixels with low ADC (<1.00 × 10 −3 mm 2 /s), assuming that lower ADC values are more relevant in assessing treatment response [8, 9]. Last, we assessed the variability in measurements for the difference in median ADC before and after neoadjuvant chemotherapy. "
    [Show abstract] [Hide abstract] ABSTRACT: The apparent diffusion coefficient (ADC) is potentially useful for assessing treatment response in nephroblastoma (Wilms tumour). However the precision of ADC measurements in these heterogeneous lesions is unknown. To assess intra- and interobserver variability of whole-tumour ADC measurements in viable parts of nephroblastomas at diagnosis and after preoperative chemotherapy. We included children with histopathologically proven nephroblastoma who had undergone MRI with diffusion-weighted imaging before and after preoperative chemotherapy. Three independent observers performed whole-tumour ADC measurements of all lesions, excluding non-enhancing areas. One observer evaluated all lesions on two occasions. We performed analyses using Bland-Altman plots and concordance correlation coefficient (CCC) calculations with 95% limits of agreement for median ADC, difference between pre- and post-chemotherapy median ADC (ADC shift) and percentage of pixels with ADC values <1.0 × 10(-3) mm(2)/s. In 22 lesions (13 pretreatment and 9 post-treatment) in 10 children the interobserver variability in median ADC and ADC shift were within the interval of approximately ±0.1 × 10(-3) mm(2)/s (limits of agreement for median ADC ranged -0.08-0.11 × 10(-3) mm(2)/s and for ADC-shift -0.11-0.09 × 10(-3) mm(2)/s). The interobserver variability for percentage of low-ADC pixels was larger and also biased. The calculated CCC confirmed good intra- and interobserver agreement (ρ-c ranging from 0.968 to 0.996). Measurements of whole-tumour ADC values excluding necrotic areas seem to be sufficiently precise for detection of chemotherapy-related change.
    Full-text · Article · May 2015
    • "Wilms' tumor (nephroblastoma) is a kidney cancer occurring most commonly in childhood, with an incidence of seven cases per 1 million children . Nephroblastoma constitutes about 6–14% of all childhood cancers, making it, after central nervous system tumors and neuroblastoma, the third most frequent solid tumor [1] [2]. The majority of Wilms tumors are diagnosed in the first decade of life. "
    [Show abstract] [Hide abstract] ABSTRACT: Background: Wilms' tumor is the most common kidney cancer in children. Treatment consists of pre- and post-operative chemotherapy, surgery and in some cases radiotherapy. The treatment of nephroblastomas is very effective. Hence, the population of adult patients cured of this cancer in their childhood is steadily growing, generating a need for longterm health assessment, including renal function, due to the specifications of the therapy and the location of the tumor. Objectives. The aim of the study was to evaluate nephrological complications after treatment for nephroblastoma. Material and Methods: The study group consisted of 50 children treated in the Department of Pediatric Hematology, Oncology and Bone Marrow Transplantation at Wroclaw Medical University (Poland) from 2002 to 2012. An analysis of the patients' medical histories was carried out. The glomerular filtration rate estimated by the Schwartz formula (GFR by Schwartz), serum creatinine levels, urea and electrolyte concentrations; the results of urinalysis and blood pressure were assessed. Each of these analyses was performed at the time of diagnosis, at the end of therapy, as well as 6 months, one year and two years after its completion. Results: The study showed that, in most cases, implemented therapy had no significant impact on the deterioration of renal parameters in the two-year period following treatment for Wilms' tumor. However, the group of patients treated with cyclophosphamide and carboplatin required more careful monitoring, due to a higher risk of renal function deterioration. Conclusions: The study shows that the problem of nephrotoxicity after treatment for Wilms' tumor is more frequent than indicated in other studies; however, the deterioration of kidney function in most cases is not serious. Additional attention should be paid to patients treated with cyclophosphamide and carboplatin. Assessment of the early and late effects of the treatment is a key element in improving the quality of the patients' life
    Full-text · Article · May 2015
    • "Patients with metastases were stratified according to the response to preoperative chemotherapy treated as in the SIOP 9 study. VP16 and carboplatin are administered for 3 days in reduced doses (VP16 of 5 Â 100 mg/m 2 has been changed to 3 Â 150 mg/m 2 and 600 mg/m 2 and CARBO to 3 Â 200 mg/ m 2 ) [15]. Naguib et al. [10] published a retrospective analysis of clinico-pathological features and treatment results for 53 previously untreated pediatric patients with WT. "
    [Show abstract] [Hide abstract] ABSTRACT: Background Wilms tumor (WT) is the most common pediatric malignant primary renal tumor. One of the main drugs used in treatment is actinomycin-D. This was not readily available in Turkey at one time. Carboplatin was used in the primary treatment of WT in order to prevent delays in treatment. The aim of this study is to present the results of patients with WT receiving carboplatin or actinomycin-D therapy.ProcedureForty-eight consecutive patients with WT treated between July 2005 and December 2011 were included in this retrospective study. The patients were treated according to Turkish Pediatric Oncology Group guidelines. Nineteen patients were treated with actinomycin-D and 29 with carboplatin (500 mg/m2/dose). The two groups were then compared in terms of 2- and 4-year overall survival (OS), event-free survival (EFS) and disease-free survival (DFS).ResultsTwo- and four-year OS rates in the carboplatin group were 90.0% and 90.0%, compared to 100.0% and 88.0%, respectively, in the non-carboplatin group. Two- and four-year EFS levels in the carboplatin group were 92.0% and 88.0%, respectively, compared to 82.0% and 76.0% in the non-carboplatin group. Two-and four-year DFS levels in the carboplatin group were 92.0% and 86.0%, respectively, compared to 77.0% and 77.0% in the non-carboplatin group.Conclusions The findings show that the carboplatin can be used as an alternative drug in the primary treatment of WT in the event that actinomycin-D is unavailable or not tolerated. Pediatr Blood Cancer © 2014 Wiley Periodicals, Inc.
    Full-text · Article · Sep 2014
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