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Outcome of Renal Transplantation for Wilms' Tumor and Denys-Drash Syndrome: A Report of the North American Pediatric Renal Transplant Cooperative Study

Jun-05

Journal Article

Authors:
van Holthe, K.; Ho, P.; Stablein, D.; Harmon, W.; Baum, M.

Secondary:
Pediatr Transplant

Volume:
9

Pagination:
305-310

URL:
http://www.ncbi.nlm.nih.gov/pubmed/15910385

Keywords:
Adolescent; Child; Denys-Drash Syndrome; Female; Graft Survival; Infant; Kidney Neoplasms; kidney transplantation; Male; Renal Dialysis; Treatment Outcome; Wilms Tumor

Abstract:
<p>In some children with bilateral Wilms&#039; tumor, reduction of tumor burden cannot be accomplished without total nephrectomy. In Denys-Drash syndrome, nephrectomy is required for associated Wilms&#039; tumor or after progression to end stage renal disease secondary to diffuse mesangial sclerosis because of risk of development of Wilms&#039; tumor. Current recommendation is to wait at least 1-2 yr after completion of chemotherapy for Wilms&#039; tumor before renal transplantation. The North American Pediatric Renal Transplant Cooperative Study dialysis (1992-2001) and transplant registries (1987-2002) were analyzed, comparing children 0-18 yr old with Wilms&#039; tumor and Denys-Drash syndrome to other primary diagnoses. There were 37 children with Wilms&#039; tumor and 33 with Denys-Drash syndrome in the dialysis registry. Of these, 10 children with Wilms&#039; tumor and three with Denys-Drash syndrome did not receive a renal transplant and all died. The cause of death was Wilms&#039; tumor in eight children with Wilms&#039; tumor and in one with Denys-Drash syndrome. The transplant registry included 43 children with Wilms&#039; tumor, 43 children with Denys-Drash syndrome, and 7469 patients with other diagnoses. Acute rejection, graft and patient survival profiles from all three groups at 6 months, 1 and 3 yr post-transplant were comparable. There were no graft failures or deaths because of recurrent Wilms&#039; tumor in the Drash group. There was one death with Wilms&#039; tumor in the Wilms&#039; group - a 2.5-yr-old child transplanted after 6 months of dialysis who died of Wilms&#039; &lt;6 months after renal transplantation. In conclusion, most children dialyzed because of Wilms&#039; tumor and Denys-Drash syndrome who did not receive a renal transplant died of Wilms&#039; tumor. However, the outcomes of children with Wilms&#039; tumor and Denys-Drash syndrome who proceeded to renal transplantation are comparable with children with other diagnoses, with no graft failures because of recurrence and only one death from Wilms&#039; tumor in a Wilms&#039; patient who received only a short course of dialysis prior to transplantation. Current practices in children with Wilms&#039; tumor and Denys-Drash syndrome appear to be on target to portend good outcome following renal transplantation.</p>

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