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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http://dx.doi.org/10.1007/s00383-003-0982-9 | DOI Listing |
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