Wilms tumor: progress to date and future considerations

Abstract

Wilms tumor is the most common tumor of renal origin found in children. In the last 50 years, remarkable progress has been made in the treatment and understanding of children with Wilms tumor. Through the development of multiagent chemotherapy and cooperative pediatric interdisciplinary groups conducting large randomized controlled clinical trials, survival has improved dramatically. In the next century it is expected that 80% of children with Wilms tumor will be long-term survivors. Therapy is progressing towards a risk-based management based not only on stage and histology but also incorporated genetic markers. This article reviews progress to date and possible future directions in the treatment of Wilms Tumor.

Primary renal botryoid rhabdomyosarcoma: diagnosis and outcome

“…Accounting for 6% of all childhood tumors, Wilm's tumor is the most common pediatric renal malignancy in more than 90% of the cases and the usual preoperative diagnosis in a child with a renal mass [6]. Other malignant tumors found in the kidney are clear cell sarcoma, rhabdoid tumor, and renal cell carcinoma [7].…”

Quality assessment for Wilms' tumor: a report from the National Wilms' Tumor Study-5

“…Currently, more then 90% of children survive 4 years after their diagnosis with 85% being cured [1,2,[10][11][12]. Nevertheless, there are populations of patients who do poorly, particularly those with locally recurrent Wilms' tumor.…”

“…Spills (13/253) were determined to be avoidable. Eight were biopsies, 5 because tumor was transected in the renal vein (4) or ureter (1). In stage II patients where lymph nodes were not sampled, there is an increase in local relapse rate that did not achieve statistical significance because of the small number of events.…”

“…The National Wilms' Tumor Study Group (NWTSG) has conducted 5 large multi-institutional clinical trials for children with Wilms' tumor [1,2]. These multidisciplinary therapeutic protocols are well established on the basis of a rich history of rigorous analysis of these prospective clinical studies.…”

Wilms' tumor, pancreatic islet cell carcinoma, and pheochromocytoma in a child

“…Wilms' tumor (WT) occurs in 1 in 10,000 children and is the most common pediatric renal malignancy with 500 cases diagnosed in the United States annually [4]. Mutation, deletion, and loss of heterozygosity at multiple chromosomal loci such as 11p13, 11p15.5, 16q3, 7p15-p11.2, and 17q12-q21 have been associated with the development of WT [5][6][7].…”