Non-ocular tumours following retinoblastoma in Great Britain 1951 to 2004.

BACKGROUND: Retinoblastoma occurs in both a heritable and a non-heritable form. In the heritable form, there is a predisposition to the development of non-ocular tumours. OBJECTIVES: To identify the types of non-ocular tumour occurring in retinoblastoma survivors and to produce estimates of risk for these tumours. METHODS: We carried out a cohort study that included 1927 cases of retinoblastoma diagnosed in Great Britain between 1951 and 2004. Cases were ascertained through the National Registry of Childhood Tumours and followed up for the occurrence of non-ocular tumours using the routine notification system based on the National Health Service Central Registers in Britain. RESULTS: Of the 1927 cases, 809 were known to have the heritable form of the disease and 1118 assumed to have the non-heritable form. 102 of the heritable and 13 of those classified as non-heritable developed a non-ocular tumour. The cumulative risk of developing such a tumour 50 years after retinoblastoma diagnosis was 48.3% (95% confidence interval: 38.1 to 59.7%) in the heritable and 4.9% (1.9 to 12.4%) in the non-heritable cases. The main categories of non-ocular tumours observed in the heritable cases were soft-tissue sarcomas (36 of which 21 were leiomyosarcoma), osteosarcoma (32), carcinoma (13), brain and central nervous system tumours (10), melanoma (9), leukaemia (4) and others (4). There were a total of 108 non-ocular tumours in 102 cases. CONCLUSIONS: There is a high risk of non-ocular tumours occurring in survivors of heritable retinoblastoma. These results have important implications for the clinical follow-up and counselling of survivors.

Patterns of care and survival for children with acute lymphoblastic leukaemia diagnosed between 1980 and 1994.

AIMS: To document survival rates after acute lymphoblastic leukaemia (ALL) during the era of modern chemotherapy, to assess effects of prognostic factors at presentation, and to investigate the relation of survival to patterns of organisation of care. PATIENTS: From a population based series of 5078 children diagnosed in the UK during 1980-94, 4988 remained for analysis after exclusion of nine children ascertained from death certificates alone and 81 who received no antileukaemia treatment. MAIN OUTCOME MEASURES: Actuarial survival rates. RESULTS: Between 1980-84 and 1990-94, the proportion of children treated at paediatric oncology centres rose from 77% to 89%, and the proportion entered into national trials rose from 59% to 82%. Each of age, sex, white blood count, immunophenotype, and Down's syndrome status had a highly significant effect on survival. Five year survival improved from 67% in 1980-84 to 81% in 1990-94, a 42% reduction in the risk of death within five years of diagnosis. Survival did not differ significantly between hospitals with different numbers of new patients per year or between paediatric oncology centres and other hospitals. Children who were entered into national trials had higher survival and this difference became greater in recent years; five year survival rates for children diagnosed during 1980-84 were 70% and 64% for trial and non-trial patients, respectively; in 1990-94 the rates were 84% and 68% for trial and non-trial patients, respectively. CONCLUSIONS: Survival after ALL continues to improve. Nearly 50 children/year diagnosed during 1990-94 survived who would have died a decade before. Survival does not vary systematically with place of treatment but is higher for children entered into national trials.

Childhood cancer in Britain: the National Registry of Childhood Tumours and incidence rates 1978-1987.

The National Registry of Childhood Tumours contains population-based data on childhood cancers diagnosed throughout Great Britain from 1962 onwards. This paper describes the methodology of the Registry, presents incidence rates for 1978-1987 and describes other uses of the data. Total age-standardised annual incidence was 118.3 per million. The most frequent diagnostic groups were leukaemias (age-standardised rate 39.8), brain and spinal tumours (27.0), lymphomas (11.1), sympathetic nervous system tumours (8.3), kidney tumours (7.7) and soft-tissue sarcomas (7.5). Incidence rates were similar to those reported from other Western industrialised countries. The data are also used for a wide range of epidemiological and other studies. These include analyses of geographical variations in incidence, trends in survival, health of long-term survivors and their offspring and the genetics of childhood cancer. Information is frequently provided for clinicians and research workers, and series of specific types of cancer are compiled for further study. The Registry depends for the completeness and accuracy of its data on a wide range of organisations and individuals, and it is essential that this cooperation continues if the Registry is to be maintained.

Survival from acute non-lymphocytic leukaemia, 1971-88: a population based study.

Survival rates were studied among 1258 children with acute non-lymphocytic leukaemia diagnosed in 1971-88 and included in the population based National Registry of Childhood Tumours. Of the total, 147 (12%) died without receiving treatment. Among the remaining treated children, actuarial five year survival rates were 6% in 1971-4, 15% in 1975-9, 23% in 1980-3, and 40% in 1984-8. Infants aged less than 1 year had a significantly worse prognosis and there was a significant trend towards lower survival rates with increasing white cell count. No independent significant effects on survival were found with sex, French-American-British (FAB) subtype, or the presence or absence of Down's syndrome. Children entered in national trials had a higher survival rate than those who were not entered, and children treated at teaching hospitals had a higher survival rate than those who were treated elsewhere. Among the 535 (43%) children who survived at least one year from diagnosis no factor studied had a significant effect on survival, emphasising the importance of achieving first remission as a determinant of long term survival.