British Journal of Urology (1992), 70,663468 01992 British Journal of Urology

Wilms’ Tumour in New Zealand 1960-1 986 B. DELAHUNT, P. B. BETHWAITE, J. N. NACEY, M. E. LEWIS and J. FRASER Departments of Pathology, Surgery and Paediatrics, Wellington School of Medicine and Health Statistical Services, Department of Health, Wellington, New Zealand

Summary-Between 1960 and 1986, 177 cases of Wilms’ tumour were reported to the New Zealand National Cancer Registry. There was an equal sex distribution and 93.2% of patients were in the paediatric age group with a mean age of 3.4 years. The mean symptom interval was 2.0 months and the majority of patients presented with localised disease, although the incidence of advanced disease was greater than that reported from other series. The 5-year survival rate for all cases was 52.5%. Data were divided into patients presenting before and after the publication of the first National Wilms’ Tumour Study (NWTS) in the United States in 1976. In patients presenting after 1976 the diagnosis was based on histology in loo%, compared to 58.6% in patients before 1977. Treatment was considered curative in a greater proportion of the post- 1976 group. Treatment modalities reflected the recommendations of the NWTS and the 5-year survival rate of this group was 78.3%, this being similar to that reported from the NWTS. Multivariate analysis showed patient age, symptom interval, tumour stage and treatment (pre- and post-NWTS) to be independent predictors of survival.

Over the last 20 years there has been considerable activity directed towards the classification and management of Wilms’ tumour (WT). In 1969 the National Wilms’ Tumour Study Group (NWTS) was established and since that time numerous reports, emanating from the 3 studies initiated by the NWTS, have detailed advances in the understanding of the behaviour and in the management of WT (Belasco and D’Angio, 1981). Specifically, the treatment protocols developed by the NWTS have resulted in a dramatic improvement in the survival of patients with this form of malignancy (D’Angio et al., 1989). The NWTS collated data from institutions in the United States and since the publication of the initial N WTS few additional reports have evaluated the clinical features and survival of patients with WT who have been treated outside that country. This study was undertaken to record the clinical characteristics and survival data of patients presenting with WT in New Zealand between 1960 and 1986. The treatment and outcome of patients Accepted for publication 7 November 1991

presenting prior to, and following, the publication of the first NWTS by D’Angio et al. (1976) were compared in order to evaluate the impact of the results of that study on the survival of patients treated in a non-collaborating centre. Patients and Methods The New Zealand Cancer Registry has operated since 1948 and has been population-based since 1960 (Foster, 1977). Malignant tumours identified in public and private hospital admissions are registered and coded according to the International Classification of Disease for Oncology (ICD) currently using the 9th edition. Registration details include patient’s age, sex, occupation, race and symptom interval (from first sign or symptom until diagnosis). Stage and grade of tumour, and aim and type of treatment are also recorded. The results from post mortem examinations and information from death certificates are abstracted by the Registry. Cancer registrations are matched with death certificate data to provide basic survival information for patients dying in New Zealand.

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All registrations classified under ICD code 189.0 (malignant neoplasms of the kidney, excluding the renal pelvis) were retrieved for the period 19601986. A major expansion in the registration record occurred in 1960 and case ascertainment was complete to the end of 1986at the time of the study. All patients diagnosed with WT were identified and the registration data were evaluated in an attempt to identify parameters of prognostic importance. The data were analysed using the Statistical Analysis System (SAS, 1988) and actuarial survival analysis was undertaken using the Kaplan-Meier method for right censored data (Kaplan and Meier, 1958). Univariate parameters were tested using the log rank test and multivariate analysis undertaken using a log-logisticmodel (Kalbfleischand Prentice, 1982). In New Zealand there were no established protocols for the management of WT before the publication of the NWTS. In 1977 the protocols of the NWTS were adopted nationally and remained in use beyond 1986, the last year of registrations in this present study. In the analysis the registrations were divided into those made prior to and following

the publication of the first NWTS report to determine the impact of that report on the outcome of WT patients in New Zealand. For the purpose of this study the pre-NWTS group was taken as all registrations prior to 1977, while the post-NWTS group was taken as all registrations after 1976.

Results Patient data During the 27-year period selected for study, 177 patients with WT were notified to the New Zealand Cancer Registry. The mean annual registration rate was 6.6 patients and annual registrations ranged from 1 patient in 1969 to 12 patients in 1984. The majority of patients (93.2%) were in the paediatric age group (< 15 years), although 12 cases were seen in adults (Fig. 1). The age-standardised incidence rate for WT in the New Zealand paediatric population for the period of study was 6.8/106 person-years. The rates for the 5-year periods commencing 1960,1965,1970,1975 and 1980 were 6.22, 6.16, 5.97, 6.19 and 8.52/106 person-years respectively. There was an equal sex distribution

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Wilms' tumour in New Zealand 1960-1986.

Between 1960 and 1986, 177 cases of Wilms' tumour were reported to the New Zealand National Cancer Registry. There was an equal sex distribution and 9...
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