ABSTRACT
In the period 1985-1995, 87 children underwent surgery for Wilms' tumour; 16 were lost to follow-up. Of the remaining children, 27 presented with stage I disease, 11 with stage II, 12 with stage III, 14 with stage IV, and 6 with stage V. One child was not staged. The histology was favourable Wilms' tumour in 44, anaplastic in 12, unclassified in 8, clear-cell sarcoma in 4, and rhabdoid tumour in 3. Although a total nephrectomy was generally performed, partial renal surgery was performed for 6 bilateral and 4 unilateral tumours, the latter including 2 fused kidneys. Preoperative chemotherapy was employed with benefit in massive tumours, tumour in fused kidneys, bilateral tumours, and preoperatively diagnosed inferior vena caval tumour thrombi. Postoperative chemotherapy, employed in all cases, consisted of actinomycin D and vincristine with the addition of adriamycin in anaplastic and advanced-stage tumours. Ten children underwent second-line chemotherapy for disease unresponsive to the above management, but only 1 of these is currently free of disease. Postoperative tumour-bed radiotherapy, used in selected cases, prevented local recurrence in stage I and II disease. However, 20% of stage I and II patients not receiving radiotherapy developed tumour-bed recurrence. Twenty-three children have died and 5 with advanced disease and incomplete follow-up are presumed to be dead. Nine children are currently on treatment; 34 have successfully completed treatment, the disease-free survival in stages I-V being 81%, 75%, 42%, 14%, and 50%, respectively. Overall disease-free survival was 69% for Wilms' tumour of favourable histology and 50% for anaplastic tumours. The 3 patients with rhabdoid tumours and 3 of 4 with clear-cell sarcomas have died. Wilms' tumour management in the developing world is compromised by cases lost to follow-up and late presentation with massive tumours and advanced stage. Preoperative chemotherapy is advantageous in a number of cases, and postoperative radiotherapy should be deployed more frequently.
