Wilms tumour: prognostic factors, staging, therapy and late effects

Abstract

Wilms tumour is the most common malignant renal tumour in children. Dramatic improvements in survival have occurred as the result of advances in anaesthetic and surgical management, irradiation and chemotherapy. Current therapies are based on trials and studies primarily conducted by large multi-institutional cooperatives including the Société Internationale d'Oncologie Pédiatrique (SIOP) and the Children's Oncology Group (COG). The primary goals are to treat patients according to well-defined risk groups in order to achieve the highest cure rates, to decrease the frequency and intensity of acute and late toxicity and to minimize the cost of therapy. The SIOP trials and studies largely focus on the issue of preoperative therapy, whereas the COG trials and studies start with primary surgery. This paper reviews prognostic factors and staging systems for Wilms tumour and its current treatment with surgery and chemotherapy. Surgery remains a crucial part of treatment for nephroblastoma, providing local primary tumour control and adequate staging and possibly controlling the metastatic spread and central vascular extension of the disease. Partial nephrectomy, when technically feasible, seems reasonable not only in those with bilateral disease but also in those with unilateral disease where the patient has urological disorders or syndromes predisposing to malignancy. Partial nephrectomy, however, is frequently not sufficient for an anaplastic variant of tumour. The late effects for Wilms tumour and its treatment are also reviewed. The treatment of Wilms tumour has been a success story, and currently in excess of 80% of children diagnosed with Wilms tumour can look forward to long-term survival, with less than 20% experiencing serious morbidity at 20 years from diagnosis. The late complications are a consequence of the type and intensity of treatment required, which in turn reflects the nature and extent of the original tumour. Continual international trial development and participation will improve matching of treatment needs with prognosis, reducing long-term complications in the majority. The advent of molecular markers of disease severity and improved functional imaging might help.

Clinical Factors in Relapses of Wilms’ Tumor – Results for the Polish Pediatric Solid Tumors Study Group

“…A retrospective analysis by Kaste et al drew attention once again to known risk factors for nephroblastoma recurrence [11]. The prognosis was worsened by unfavorable histopathology with diffuse anaplasia, which were associated with lower sensitivity to chemotherapy.…”

Topotecan distribution in an anephric infant with therapy resistant bilateral Wilms tumor with a novel germline WT1 gene mutation

“…It has been suggested that certain germline WT1 gene mutations are associated with a more resistant phenotype, which in bilateral cases harbors a higher risk for an eventual anephric status [14,26]. In this case report we describe a 10-month-old African girl with chemotherapy un-responsive bilateral Wilms tumor, with a novel germline WT1 gene splice site mutation in intron 6, who ultimately, underwent sequential bilateral tumor-nephrectomy.…”

Local behavior and lymph node metastases of Wilms' tumor: accuracy of computed tomography

“…However, it is difficult to determine the respectability of a tumor (3) . Tumor histology and staging represent the main prognostic factors in cases of WT (4) . The tumor staging is defined by surgical and histopathological findings.…”

“…Currently, two approaches for the initial management of WT are adopted with similar results (3,4) . Such approaches are based on studies developed by two cooperative groups, namely, National Wilms' Tumor Study (NWTS) and International Society of Paediatric Oncology (SIOP).…”