Adult Wilms tumour.

BACKGROUND: Wilms tumour is the most common renal tumour in children, accounting for above 90% of cases and comprising 8% of paediatric solid tumours. In adults, it is extremely rare and represents only 0.5% of all renal tumours. We reviewed 9 patients older than 16 years: three were treated in the Department of Paediatric Surgery and three in the Department of Urology; additionally, 3 patients were referred to us for consultation with regard to further treatment after having been operated on previously in other centres. Clinical presentation, stage distribution, histology, treatment, and prognosis of all the patients are presented. PATIENTS AND METHODS: The age of 9 patients ranged from 17 to 32 years: there were 8 men and 1 women. Tumour stages were defined according to SIOP classification: 4 were of local stage II, 3 of stage III, and 2 of stage IV with metastases to lungs and bones. Three of them were treated according to the paediatric protocol of the Society of Paediatric Oncology (SIOP 93-01 protocol)-all after a fine-needle biopsy which confirmed nephroblastoma, received preoperative chemotherapy of two drugs (VCR + ACT D) during 4 weeks. Histological studies revealed a low-risk cystic tumour in one case, intermediate-risk tumour in 2 patients, and high-risk tumour with blastemal predominance or with the presence of diffuse anaplasia in 5 patients. One tumour was defined as a mixed high-risk tumour: renal cell carcinoma with nephroblastoma of blastemal predominance. RESULTS: Complete remission was achieved in 3 patients. Three relapsed after complete remission of 6-30 months. A second-line therapy was undertaken in these patients as well as in those who had undergone the initial treatment elsewhere. But the second-line therapy proved ineffective. CONCLUSIONS: Adult patients with Wilms tumour have more aggressive histology and poorer prognosis than children.