Renal transplantation in the management of bilateral Wilms' tumour (BWT) and of Denys-Drash syndrome (DDS).
BACKGROUND
Wilms' tumour (WT) occurs bilaterally in approximately 5-7% of affected children. In some patients, complete surgical removal of the malignant tissue cannot be achieved without bilateral total nephrectomy. In Denys-Drash syndrome (DDS), bilateral nephrectomy is indicated both because of the associated nephropathy usually progressing rapidly to end-stage renal failure and because of the high risk of WT development in any residual renal tissue.


METHODS
Case records of patients with a diagnosis of either bilateral WT (BWT) or DDS, who underwent bilateral nephrectomy and subsequent renal transplantation between 1980 and 1996 at the Hospital for Sick Children, London, were reviewed.


RESULTS
Allogeneic renal transplantation was performed in two children with BWT and four with DDS, three of whom had developed unilateral WT by the time their kidneys were removed. Renal transplantation was performed 15-49 months after bilateral nephrectomy at a mean age of 45 (26-76) months, with a minimum of 1 year tumour-free survival after completion of chemotherapy in those with WT. One patient died after renal transplantation. Five children had a favourable outcome, with a mean follow-up of 80 (29-121) months post-renal transplantation.


CONCLUSIONS
Advances in dialysis and transplantation programmes for young children offer the potential for a marked improvement in the prognosis for patients with BWT and for those with DDS.
Rudin
C
C
Department of Nephrology, Great Ormond Street Hospital for Children NHS Trust, London, UK.
Pritchard
J
J
Fernando
O N
ON
Duffy
P G
PG
Trompeter
R S
RS
D002675:Child, Preschool; D005260:Female; D005835:Genitalia / Q000002:abnormalities; D006801:Humans; D007223:Infant; D007674:Kidney Diseases / Q000235:genetics / Q000601:surgery*; D007680:Kidney Neoplasms / Q000235:genetics / Q000601:surgery*; D016030:Kidney Transplantation; D008297:Male; D009378:Neoplasms, Multiple Primary / Q000235:genetics / Q000601:surgery*; D009392:Nephrectomy; D013577:Syndrome; D009396:Wilms Tumor / Q000235:genetics / Q000601:surgery*