Corrado Magnani

Childhood leukaemia in Europe after Chernobyl: 5 year follow-up

The European Childhood Leukaemia - Lymphoma Incidence Study (ECLIS) is designed to address concerns about a possible increase in the risk of cancer in Europe following the nuclear accident in Chernobyle in 1986. This paper reports results of surveillance of childhood leukaemia in cancer registry populations from 1980 up to the end of 1991. There was a slight increase in the incidence of childhood leukaemia in Europe during this period, but the overall geographical pattern of change bears no relation to estimated exposure to radiation resulting from the accident. We conclude that at this stage of follow-up any changes in incidence consequent upon the Chernobyl accident remain undetectable against the usual background rates. Our results are consistent with current estimates of the leukaemogenic risk of radiation exposure, which, outside the immediate vicinity of the accident, was small.

Multicentric study on malignant pleural mesothelioma and non-occupational exposure to asbestos.

Insufficient evidence exists on the risk of pleural mesothelioma from non-occupational exposure to asbestos. A population-based case–control study was carried out in six areas from Italy, Spain and Switzerland. Information was collected for 215 new histologically confirmed cases and 448 controls. A panel of industrial hygienists assessed asbestos exposure separately for occupational, domestic and environmental sources. Classification of domestic and environmental exposure was based on a complete residential history, presence and use of asbestos at home, asbestos industrial activities in the surrounding area, and their distance from the dwelling. In 53 cases and 232 controls without evidence of occupational exposure to asbestos, moderate or high probability of domestic exposure was associated with an increased risk adjusted by age and sex: odds ratio (OR) 4.81, 95% confidence interval (CI) 1.8–13.1. This corresponds to three situations: cleaning asbestos-contaminated clothes, handling asbestos material and presence of asbestos material susceptible to damage. The estimated OR for high probability of environmental exposure (living within 2000 m of asbestos mines, asbestos cement plants, asbestos textiles, shipyards, or brakes factories) was 11.5 (95% CI 3.5–38.2). Living between 2000 and 5000 m from asbestos industries or within 500 m of industries using asbestos could also be associated with an increased risk. A dose–response pattern appeared with intensity of both sources of exposure. It is suggested that low-dose exposure to asbestos at home or in the general environment carries a measurable risk of malignant pleural mesothelioma.

Parental occupation and other environmental factors in the etiology of leukemias and non-Hodgkin's lymphomas in childhood: a case-control study.

We report the results of a hospital-based, case-control study on acute lymphocytic leukemia (ALL), acute non-lympocytic leukemia (AnLL) and non-Hodgkin lymphoma (NHL) in childhood. The study was conducted from 1981 to 1984 in Turin (Italy). One hundred and forty-two children with ALL, 22 with AnLL and 19 with NHL were included, as well as 307 controls. Information on parental smoking habits, parental occupation, ionizing radiation and childhood diseases were collected using a standard questionnaire during a personal interview of the relative attending the child in the hospital. The odds ratios for antenatal diagnostic radiation were 1.1 (NS) for ALL and 2.4 (NS) for AnLL. No association was found with diseases in childhood. Paternal and maternal smoking habits were similar for ALL cases and controls. Both maternal and paternal smoking were associated with NHL: for paternal smoking, odds ratios were around 5, but without a correlation with number of cigarettes. Positive associations observed with maternal employment were: ALL with teacher and cleaner; AnLL and textile worker; NHL and baker. Corresponding association with paternal jobs were: ALL with clerks, farmers and employment in office equipment production; AnLL and workers in building, tire or textile industries; NHL and lorry drivers, workers in the building or in the wood and furniture industry.

Accuracy of death certificate diagnosis of amyotrophic lateral sclerosis.

STUDY OBJECTIVE--The aim was to determine the reliability of official mortality statistics in estimating long term trends of amyotrophic lateral sclerosis (ALS) in Italy. DESIGN--The study was a mortality follow up of cases of ALS. SETTING--Piedmond Region, northern Italy. SUBJECTS--Cases of ALS were identified from multiple sources between 1966 and 1985. MAIN RESULTS--Cause of death was determined for the cases who died between 1970 and 1985. Death certificates were obtained in 488 out of 510 cases (95.7%). ALS was mentioned in 365 (74.8%) of cases. The most frequent erroneous diagnoses were multiple sclerosis and malignant tumours. Demographic variables, such as sex, age at death, province of death, and calendar year of death, did not influence the percentage of true positive cases significantly. CONCLUSIONS--The death certificate diagnosis of ALS appears to be adequate for use in descriptive and analytical epidemiology.

Childhood leukaemia following the Chernobyl accident: the European Childhood Leukaemia-Lymphoma Incidence Study (ECLIS).

The objective of the European Childhood Leukaemia-Lymphoma Incidence Study (ECLIS) is to investigate trends in incidence rates of childhood leukaemia and lymphoma in Europe, in relation to the exposure to radiation which resulted from the accident at the Chernobyl nuclear power plant in April 1986. In this first report, the incidence of leukaemia in children aged 0-14 is presented from cancer registries in 20 European countries for the period 1980-1988. Risk of leukaemia in 1987-1988 (8-32 months post-accident) relative to that before 1986, is compared with estimated average dose of radiation received by the population in 30 geographic areas. The observed changes in incidence do not relate to exposure. The period of follow-up is so far rather brief, and the study is planned to continue for at least 10 years.

Survival of children with neuroblastoma: time trends and regional differences in Europe, 1978–1992

Neuroblastoma is one of the most common solid cancers in children. We present the data collected for the EUROCARE II study, describing survival patterns for children diagnosed in Europe 1985--1989 in detail, and exploring time trends from 1978 to 1992. On average, the mean 5-year survival rate was considerably higher in infants (79%) compared with older children (30--33%). The risk of death has dropped by 37% from 1978--1981 to 1990--1992. There is a pronounced difference between countries, with Scotland and England and Wales having two of the lowest survival rates (28% (95% confidence interval (CI) 14--48) and 36% (95% CI 31--41) 5-year survival rates, respectively). The survival rates in France, Germany and Italy (48--66% 5-year survival rate) were among the highest. This pattern corresponds to the incidence rates for these countries. It can be assumed that in neuroblastoma, both incidence and survival are related to the frequency of diagnosing asymptomatic cases with good prognosis among infants. However, one cannot ignore possible intercountry differences in the effectiveness of therapy.

Variation in survival of children with central nervous system (CNS) malignancies diagnosed in Europe between 1978 and 1992: the EUROCARE study.

EUROCARE is a population-based survival study including data from European Cancer Registries. The present paper analyses survival after a malignant neoplasm of the central nervous system (CNS) in childhood (aged 0--14 years at diagnosis). The database includes 6130 cases from 34 population-based registries in 17 countries: 1558 were primitive neuroectodermal tumours (PNET) and 4087 astrocytoma, ependymoma or other gliomas: these morphologies were grouped in the analyses in order to reduce the diagnostic variability among the registries. 87% of cases were microscopically diagnosed (range among registries 71--100%) and losses to follow-up were limited to 2% (range 0--14%). Actuarial analyses indicate that the European (weighted) average of 5 years cumulative survival for cases diagnosed in 1978--1989 was 53% (95% confidence interval (CI) 49--57) for CNS neoplasms, 44% (95% CI 37--50) for PNET and 60% (95% CI 55--65) for the glioma-related types. Analysis of the sub-set of cases diagnosed in 1985--1989 revealed better results: cumulative survival at 5 years was 61% (95% CI: 55--65) for all CNS neoplasms; 48% (95% CI 41--56) for PNET and 68% (95% CI 62--73) for glioma-related types. Compared with older children, infants showed poorer prognosis: in 1978--1989 the 5-year survival rate was 33% (95% CI 23--45) and in 1985--1989 it was 46% (95% CI 34--59). Variability among countries was very large, with 5-year survival for CNS tumours diagnosed in 1985--1989 ranging from 28% in Estonia (95% CI 17--43) to 73% Sweden (95% CI 59--83) and 75% in Iceland (95% CI 35--95) and 73% in Finland (95% CI 66--79). Time trends were studied in a multivariate analysis observing a reduction in the risk of death in periods of diagnosis 1982--1985 (hazard ratio (HR)=0.85; 95% CI 0.78--0.93) and 1986--1989 (HR=0.70; 95% CI 0.64--0.77) compared with 1978--1981. The analysis were extended to 1990--1992 for the countries whose registries provided data for that period did not indicate any further progress. Results of this study confirm the large variability in European countries and indicate a positive trend in the survival probability for cases diagnosed in the 1980s.

Amyotrophic lateral sclerosis mortality in Italy, 1958 to 1987 A cross‐sectional and cohort study

We analyzed the mortality rates of amyotrophic lateral sclerosis (ALS) in Italy from 1958 to 1987. The overall mean annual mortality rate, adjusted to the 1981 Italian population, was 0.68/100,000 (95% confidence interval, 0.64 to 0.72); 0.86 for men; and 0.62 for women. During that period, mortality increased for both sexes: from 1958/1962 to 1983/1987, mortality increased by 60% for women and 24% for men. The slope for women was 0.07, and for men, 0.02. Moreover, the mortality rate increased in older age groups and was stable for subjects under 45 years old. There was no clear birth-cohort effect, but in any cohort the age-specific mortality rate increased with advancing age, with no decline for the oldest age. Based on these data, the increase of ALS mortality rates is probably due to the effect of changing methodologic and demographic variables and the decrease of mortality of some competing diseases, rather than a real rise of the risk in the whole population or in specific birth cohorts.

Physical impairment and social life goals among adult long-term survivors of childhood cancer: a population-based study from the childhood cancer registry of Piedmont, Italy.

Aims and Background The study describes the health status and the attainment of life goals in the adult survivors of childhood cancer recorded at the Childhood Cancer Registry of Piedmont. Methods and Study Design A postal questionnaire was sent to the general practitioner of the 690 cases born before 1976 and alive in 1991 after at least 5 years from diagnosis. The answer was received for 485 (72.9%) included in the analyses. Items in the questionnaire were: sequelae related to cancer and its treatment, health-related quality of life (according to Blooms criteria), educational level attained, and employment status. Results Vital and marital status were obtained for all 690 cases at the offices of the town of residence. No medical condition was reported for 309 cases (63.7%). The overall proportion with a high school or university education was compared to corresponding figures for Piedmont in 1991, adjusted by age, and was as high as in the general population. Similar results are observed for occupation. Patients of both genders were married less than expected. Patients with leukemia (112 cases), non-Hodgkins lymphoma (34) or Hodgkins lymphoma (52) were reported to have the highest quality of life. In contrast, patients with tumors of the central nervous system (151) had the highest frequency of sequelae and the lowest score for health-related quality of life. They also presented the lowest educational achievement, the lowest proportion of employment and, among males, the lowest frequency of marriage. Conclusions Our study shows a good social adjustment of adult survivors from childhood cancer, with the exception of central nervous system tumors. From the methodologic point of view, the present study shows the feasibility of surveillance surveys on health-related quality of life with the contribution of general practitioners.

Childhood malignancies in the EUROCARE study: the database and the methods of survival analysis

This paper describes the database of children with cancer in the EUROCARE study and the methodology used to analyse and report survival. This is the first systematic evaluation of survival after childhood cancer on a large scale in Europe: approximately 45,000 cases were included, diagnosed between 1978 and 1992 (34,814 cases diagnosed in 1978--1989 and an additional set of 9495 cases diagnosed in 1990--1992) and followed-up until 1995. Data were provided from 34 population-based registries (four specialised for childhood cancer registrations and one specialised registry for childhood leukaemia) in 17 countries of Europe (where there was national coverage in 10 countries). Quality of the data was fairly good, given the general differences among the countries and their health systems, thereby allowing for comparisons between them. Among cases diagnosed in 1978--1989, overall 2.0% were lost to follow-up, 91.8% were microscopically diagnosed and 93.4% of alive cases had at least 5 years of observation. Survival proportions (observed survival) were calculated for each of the countries involved, by age group (0, 1--4, 5--9, 10--14 years), gender, different time periods and selected diagnostic groups. Age-standardised cumulative survival rates and European averages (weighted and pooled) were also computed. Cox regression models were used to evaluate geographical and temporal differences. The EUROCARE database represents a unique source of information on survival of childhood cancer patients in Europe, intercountry differences and time trends in survival.