Background: Wilms' tumour is the most common primary renal tumour of childhood. The aim or the study was to evaluate the results of treatment in patients with unfavourable histology Wilms' tumour.
Material and methods: 44 children were treated for nephroblastoma (Wilms' tumour) according to the SIOP 93-01 protocol in the Department of Paediatrics. Haematology. Oncology and Endocrinology, Medical University of Gdansk, between 1993 and 2002. Eleven children were classified as having tumours with high-grade malignancy. Among these 2 cases of nephroblastoma with diffuse anaplasia, 6 with sarcomatous stroma and 2 cases with clear cell sarcoma of the kidney were diagnosed. In one patient file tumour structure was undefined due to complete necrosis of the tumour tissue. The staging stratification was as follows: stage I-l, II N (-)-l, II N (+) -3, III- 6, including a neoplasmic occluding thrombus in the inferior vena cava, IV - 0, V-0. All children were treated according to the SIOP 93-01 protocol.
Results: complete remission was achieved in 9 patients. Two children died. Fatal outcome was attributed in one case to cancer progression (primary resistance to chemotherapy after 14 months of treatment) and in the other, to the treatment complications (fungal sepsis after undergoing the third course of chemotherapy Carbo+ VP-16). In the analysed group there were no patients with stage IV disease, no relapses were observed.
Conclusions: our observations support the opinion, that high-grade malignancy histology is the basic unfavourable prognostic factor in nephroblastoma.