Advances and changes in the treatment of children with nephroblastoma

Department and Clinic of Pediatric Oncology, Haematology and Bone Marrow Transplantation, Wroclaw Medical University, Poland.
Advances in Clinical and Experimental Medicine (Impact Factor: 1.1). 11/2012; 21(6):909-820.
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.

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    • "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. "
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    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
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    ABSTRACT: A nephroblastoma is a tumor arising from metanephric blastema occurring in childhood. Among laboratory rodents, nephroblastoma has been frequently reported in rats, but it remains exceedingly rare in mice. The present work describes a nephroblastoma in a young mouse homozygous for the specific Trp53 R172H point mutation coupled with targeted deletion of the Pin1 gene. The affected kidney was effaced by a biphasic tumor with an epithelial component arranged in tubules surrounded by nests of blastemal cells. Immunohistochemically, the neoplasm was diffusely positive for Wilms' tumor antigen. The epithelial component expressed markers of renal tubular differentiation including wide-spectrum cytokeratin, E-cadherin and folate-binding protein. Furthermore, the neoplasm exhibited a high proliferative index and diffuse nucleocytoplasmic β-catenin expression. Based on histological and immunohistochemical features, a diagnosis of nephroblastoma potentially associated with Trp53 loss and oncogenic β-catenin activation has been proposed.
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