ABSTRACT
Bilateral Wilms'tumor (BWT) affects approximately 5% of children with WT. The goal of treatment in BWT is to preserve normal renal function as much as possible, in order to postpone and reduce the risk of end-stage renal disease and other long-term complications. For neoadjuvant chemotherapy, vincristine/dactinomycin/doxorubicin is used in cases with favorable histology. Cases with diffuse anaplasia or other unfavorable histology receive an intensified regimen, including additional agents such as ifosfamide, cy-clophosphamide, carboplatin, and others. Nephron-sparing surgery is performed to preserve long-term renal function. Radiotherapy is given according to local staging, when margins or lymph nodes are positive, or tumor spillage has occurred, or pathological type is ana-plasia. The survival rate of relapsed BWT is low. Some experts suggest that patients with recurrent BWT are most likely to benefit from high-dose therapy including stem-cell transplantation, but this remains controversial.
ABSTRACT
Object To investigate the correlation of IGF-2 exon 9 CpG island methylation and the expression of IGF-2,and further explore the mechanism of Wilms tumor. Methods The IGF-2 exon 9 methylation status and IGF2 gene expression in 42 cases of Wilms tumor and corresponding normal tissues were detected by using methylation-sensitive restriction enzyme PCR and allele-specific IGF2 gene expression analysis.Results IGF-2 exon 9 methylation rate was 15% in W ilms tumor group, and 97% in normal tissue group. The difference was significant (P < 0.01), and the difference in pathological type was also significantly different (P < 0.01). Exon 9 unmethylated tissues showing biallelic expressions of IGF-2 were significantly higher than that of methylated tissues (P < 0.05). Conclusion IGF-2 exon 9 unmethylation may be the cause of biallelic expression of IGF, which contritues to tumorigenesis in Wilms tumor.
ABSTRACT
OBJECTIVES: The aim of this retrospective study is to compare surgical complications and long-term survival in children with Wilms' tumor (WT) and tumor thrombus receiving or not preoperative chemotherapy MATERIALS AND METHODS: Review of the charts of 155 children with WT treated between 1983 and 2005, and analysis of 16/155 (10.3 percent) children with WT who presented cavoatrial tumor extension, being 8/16 IVC and 8/16 atrial thrombus RESULTS: Median age was 54 months. 2/16 had cardiac failure as the first symptom. 11/16(7 IVC and 4 atrial extension) (67 percent) were submitted to preoperative chemotherapy with vincristine plus actinomycin D, and 5/16(1 IVC and 4 atrial) (33 percent) underwent initial nephrectomy and thrombus resection. So, 11 patients were submitted to preoperative VCR/ACTD and 2/11 (18.1 percent) had complete regression of the thrombus, 6/11(54.5 percent) partial regression and 3/11 (27 percent) had no response. Among the partial responders, nephrectomy with thrombus removal was performed in all, including one patient with previous intracardiac involvement, without extracorporeal circulation procedures. In two of the three non-responders, cardiopulmonary bypass was necessary for thrombus removal. There were no surgical related deaths. Long-term survival is 91 percent in the group submitted to preoperative chemotherapy and 100 percent in the group who had surgery as first approach CONCLUSION: Preoperative chemotherapy was able to reduce thrombus extension in 8/11 (73 percent) treated patients and cardiopulmonary bypass was avoided in 2 patients with atrial thrombus. Surgical resection of tumor and thrombus was successful in all cases, receiving or not preoperative chemotherapy and overall survival was similar in both groups.