[Nephroblastoma in children aged less than 6 months at diagnosis].

UNLABELLED

We present the results of treatment of kidney tumours in newborns and infants aged less than 6 months, in the years 1993-2000, from the Nephroblastoma Committee of the Polish Paediatric Group of Solid Tumours (PPGGL). We have analysed the diagnostic and treatment results in the group of 31 children aged 0 to 6 months. For 19 children registered between 1993 and 1996, event-free survival (EFS) and overall survival (AS) were assessed. Among 450 children registered between 1993 and 2000 by PPGGL and treated for kidney tumours, there were 31 (7.1%) newborns and infants aged below 6 months. The accuracy of diagnosis based on imaging studies was 97%. Only in one child the initial diagnosis of kidney tumour was not confirmed; cystic degeneration of kidney was finally established. The tumours removed during surgery were small, with average size 213 cm3, and in half of the cases the size of the tumour did not exceed 165 cm3. Primary complete excision of the tumour was performed in 21 children (67.7%). In 10 cases histopathology confirmed mesoblastic nephroma, in 19 cases nephroblastoma and in 2 cases sarcoma clarocellulare. In 10 infants (32.2%) with nephroblastoma delayed surgery preceded by chemotherapy was performed. Indications for initial preoperative chemotherapy comprised: tumour in a single kidney, tumour in a horseshoe kidney, preoperative diagnostic biopsy of the tumour and large tumour in neonates older than 3 months. In almost 70% of the children the stage of advancement was low (stage I and IIN-). Histopathology of excised tumours confirmed in 42% of cases low risk, and in 51.6% intermediate risk. Intraoperative complications occurred in 5 infants (16%). The tolerance of reduced chemotherapy by the infants was good. AS was 100%. ESF for the 19 children registered for nephroblastoma between 1993 and 1996 for all stages of advancement and types of histology was 94.75%.

CONCLUSIONS

1. Mesoblastic nephroma and low risk nephroblastoma are the most common tumours in children within the first three years of life. 2) The results of treatment of nephroblastoma in the youngest children (below 6 months of age) are the most favourable and represent world standards.3) Surgical complications in children operated primarily for nephroblastoma indicate the need of performing such operations in academic centres, specialised in newborn surgery. 4) In infants with extensive kidney tumours older than 3 months, primarily considered as inoperative, individual induction chemotherapy should be taken into account.