Abstract
More than 5000 cases of malignant renal tumour diagnosed in children under the age
of 15 years during the period 1978–1997 in Europe, were extracted from the database
of the Automated Childhood Cancer Information System (ACCIS). In 1988–1997 the age-standardised
incidence rate of childhood renal tumours in Europe was 8.8 per million, with significant
differences between regions. Wilms’ tumour (WT, M-8960) accounted for 93% of renal
tumours and about 7% were bilateral. The incidence rates of WT increased over the
20 years, by 0.7% per year. European 5-year survival for children diagnosed with WT
in 1988–1997 was 85%, ranging from 73% in the East to 91% in the North. Patients in
the age group 0–3 years at diagnosis had a more favourable prognosis (5-year survival
87%) than those diagnosed later (81%), P < 0.0001. Patients with unilateral WT (n = 2085) had better 5-year survival (85%) than 154 patients with bilateral tumours (76%),
P = 0.003. Five-year survival for 64 patients with clear cell sarcoma of kidney was 68%,
for 43 patients with rhabdoid tumour of kidney it was 23%, and for 56 patients with
renal cell carcinoma it was 87%. For combined European data, 5-year survival for WT
increased from 73% in 1978–1982 to 87% in 1993–1997 and the increase was significant
in three out of five regions (East, North and West). Further development and exploitation
of the ACCIS database will benefit clinical management and aetiological studies.
Keywords
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