RENAL-TRANSPLANTATION IN THE MANAGEMENT OF BILATERAL WILMS-TUMOR (BWT) AND OF DENYS-DRASH-SYNDROME (DDS)

Background. Wilms' tumour (WT) occurs bilaterally in similar to 5-7% o f affected children. In some patients, complete surgical removal of th e malignant tissue cannot be achieved without bilateral total nephrect omy. In Denys-Drash syndrome (DDS), bilateral nephrectomy is indicated both because of the associated nephropathy usually progressing rapidl y to endstage renal failure and because of the high risk of WT develop ment in any residual renal tissue. Methods. Case records of patients w ith a diagnosis of either bilateral WT (BWT) or DDS, who underwent bil ateral nephrectomy and subsequent renal transplantation between 1980 a nd 1996 at the Hospital for Sick Children, London, were reviewed. Resu lts. Allogeneic renal transplantation was performed in two children wi th BWT and four with DDS, three of whom had developed unilateral WT by the time their kidneys were removed. Renal transplantation was perfor med 15-49 months after bilateral nephrectomy at a mean age of 45 (26-7 6) months, with a minimum of 1 year tumour-free survival after complet ion of chemotherapy in those with WT. One patient died after renal tra nsplantation. Five children had a favourable outcome, with a mean foll ow-up of 80 (29-121) months post-renal transplantation. Conclusion. Ad vances in dialysis and transplantation programmes for young children o ffer the potential for a marked improvement in the prognosis for patie nts with BWT and for those with DDS.