Thirty‐year population‐based review of childhood renal tumours with an assessment of prognostic features including tumour DNA characteristics

Abstract

We have reviewed all paediatric kidney tumours seen in the West Midlands Health Authority Region over a 30-year period. There were 205 cases confirmed after a review of the pathology by three paediatric pathologists. Seven were cases of bone metastasising renal tumour (clear cell sarcoma), 5 were rhabdoid tumours, 2 were renal cell carcinomas, and 13 were mesoblastic nephromas. In 3 cases, it was not possible to define further the histological diagnosis. The remaining 175 cases were considered to be Wilms' tumour (86%), which is equivalent to an incidence of 5.7/10(6)/year. In the cases of Wilms' tumour, there were 91 boys and 84 girls (1.1:1). The majority of patients were Caucasian with only 7% of non-Caucasian origin. At presentation, 78% of the patients were less than 5 years old. All of these patients except 9 had surgery as part of their treatment, 154 children had total nephrectomy, 3 had partial nephrectomy, and 9 had other surgical procedures. The majority also received chemotherapy and radiotherapy. Sex, chemotherapy, and stage all had prognostic significance in univariate analysis. The actuarial survival at 10 years increased from 17% for patients treated in the first decade of the study to 78% for patients treated in the third. DNA characteristics were investigated using flow cytometry in paraffin-embedded material and adequate information was obtained in 73 cases of Wilms' tumour. Only 7 had aneuploid tumours. Univariate survival analysis of these 73 results showed that stage, sex, the percentage of cells in the synthetic phase and the proliferative index from the DNA investigations had predictive value.

DNA ploidy and proliferative activity (S-phase) in childhood soft-tissue sarcomas: their value as prognostic indicators

“…n ju -mm., mj m recognised that in subgroups of ALL (Look et al, 1985) and neuroblastoma (Look et al, 1984;Huddart et al, 1992), hyperdiploid tumour stem lines seem to favour prognosis compared with their diploid or tetraploid counterparts, whereas this prognostic feature seems to be reversed in Wilms' tumour and most adult tumours (Douglass et al, 1986;Schmidt et al, 1986;Merkel et al, 1987;Barrantes et al, 1993). Data from other tumours such as medulloblastoma and hepatoblastoma are conflicting (Yasue et al, 1989;Hata et al, 1991;Zerbini et al, 1993).…”

Renal cell carcinoma with MiTF/TFE3 translocation in children: Report of a case at the stage of lymph node involvement

“…[3,4] Indeed, it represents only 2-6% of renal tumours in children against 85-87% for nephroblastoma. [1][2][3]9,10] In literature, most publications on RCC in children are clinical cases or small series. [10][11][12][13] To our knowledge, this is the first case reported in Senegal.…”

“…Indeed, only 0.3-1.3% of RCCs occur during childhood, representing 2-6% of renal tumours in children against 85-87% for nephroblastoma. [1][2][3] Unlike nephroblastoma, RCC is resistant to chemotherapy and radiotherapy. [4] Treatment occurs mainly through surgical removal of the tumour.…”

Diagnosis and Treatment of Renal Cell Carcinoma in Children: A Report from the Polish Pediatric Rare Tumor Study Group

“…Discussion ▼ RCC usually occurs between the fourth and sixth decades of life, predominantly in males [2] . In our study cohort resulting from a survey to all Polish pediatric oncological departments, the median age at diagnosis of the 21 registered patients was 12 years, predominantly females were aff ected.…”