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Relapses in Wilms tumour
1Division of Pediatric Surgery, Department of Pediatrics, University Hospital, 221 85 Lund, Sweden. carl-magnus.kullendorff@skane.se
Insights
Relapse in Wilms tumour (WT) is linked to high stage and unfavorable histology. While chromosome aberrations were noted, only 16q deletions strongly correlated with metastasis in this pediatric cancer study.
Area of Science:
- Pediatric Oncology
- Cancer Genetics
- Clinical Research
Background:
- Wilms tumour (WT) is a common pediatric kidney cancer.
- Understanding relapse patterns and prognostic factors is crucial for improving outcomes.
- Previous studies suggest links between clinical factors and WT recurrence.
Purpose of the Study:
- To analyze relapse patterns in a cohort of children treated for Wilms tumour.
- To identify clinical and cytogenetic factors associated with WT relapse and survival.
- To investigate the role of specific chromosomal aberrations in WT metastasis.
Main Methods:
- Retrospective analysis of 54 children treated for Wilms tumour over 18 years.
- Review of clinical data including diagnosis stage, histology, and relapse sites.
- Karyotypic analysis to detect clonal chromosome aberrations in tumor samples.
Main Results:
- Thirteen children (24%) experienced relapse, with a mean time to first relapse of 17 months.
- High stage at diagnosis, unfavorable histology, and nephroblastomatosis were associated with relapse.
- Tumors with deletions of chromosome arm 16q consistently metastasized; no other significant karyotypic correlations with outcome were found.
Conclusions:
- High stage and unfavorable histology are key indicators for relapse risk in pediatric Wilms tumour.
- Specific chromosomal aberrations, particularly 16q deletions, may predict metastatic potential in WT.
- Further research into the genetic underpinnings of WT relapse and metastasis is warranted.
Abstract:
During an 18-year period, 54 children were treated for Wilms tumour (WT). Thirteen of them, 6 boys and 7 girls, had a relapse. Mean age at diagnosis was 50 months, range 5-233 months. The investigations revealed stage I in 5 cases, stage II in 2, stage III in 3, stage IV in 1 and stage V in 2.The histology was favourable in 6 cases, intermediate in 4 and unfavourable in 3. Clonal chromosome aberrations were detected in 8 cases. The mean time to first relapse was 17 months, range 1-76 months. The location was the local region in 3 cases, other kidney in 3, lungs in 7 and skeletal in 2. A second relapse occurred in 6 patients and a third relapse in 2 patients. Seven patients died after a mean follow-up of 84 months, range 42-180 months, from primary treatment. The 6 surviving children had no evidence of disease after a mean follow-up of 70 months, range 42-120 months, after treatment ended. High stage at diagnosis, unfavourable histologic subtype and occurrence of nephroblastomatosis were found not only in the children with relapse. No significant correlations were found when comparing the karyotypic features with clinical outcome. All 3 tumours with deletions of chromosome arm 16q metastasized.
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