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Biomedical subjects

C A Stiller

Publications and source records attributed to C A Stiller.

At least 55 records · Page 3Linked to original sources

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.

Adolescent↗

Population based survival rates for childhood cancer in Britain, 1980-91.

OBJECTIVES: To investigate the survival of children with cancer diagnosed during 1980-91 in order to assess the impact of developments in medical care on a population basis. DESIGN: Retrospective cohort study. SETTING: Great Britain. SUBJECTS: 14973 children with cancer diagnosed during 1980-91 and included in the population based National Registry of Childhood Tumours. MAIN OUTCOME MEASURES: Actuarial survival rates. RESULTS: For all cancers combined, two year survival increased from 66% to 76% between 1980-2 and 1989-91, and five year survival increased from 57% to 65% between 1980-2 and 1986-8. Significant increases in survival rates occurred among children with acute lymphoblastic leukaemia, acute nonlymphocytic leukaemia, retinoblastoma, osteosarcoma, Ewing's sarcoma, rhabdomyosarcoma, and malignant gonadal germ cell tumours. No trend in survival was seen for children with Hodgkin's disease, central nervous system tumours, neuroblastoma, or Wilms's tumour. CONCLUSIONS: Nearly two thirds of children who have cancer diagnosed can now expect to survive at least 10 years.

Adolescent↗

Centralised treatment, entry to trials and survival.

A review was carried out of the published literature on survival rates for cancer in relation to patterns of organisation of medical care, specifically treatment at specialist centres or at hospitals treating larger numbers of patients and treatment by protocol, usually within the context of a clinical trial. Centralised referral or entry to trials was frequently associated with a higher survival rate, particularly for the less common cancers, and was never found to be associated with a lower survival rate. Few studies were identified for any one cancer site and some antedated current methods of treatment. At a time when the health service in the United Kingdom is undergoing far-reaching organisational change, further research is needed to establish the most beneficial patterns of care for people with cancer. Population-based cancer registries are an invaluable source of data for such studies.

Cancer Care Facilities↗

Neurofibromatosis and childhood leukaemia/lymphoma: a population-based UKCCSG study.

There is a well-known raised risk of leukaemia in children with neurofibromatosis type 1 (NF-1). We carried out the first detailed population-based study of leukaemia and non-Hodgkin lymphoma (NHL) associated with NF-1 in order to estimate the risk and elucidate the relationship between these conditions. Over the 17 year study period there were five cases of chronic myelomonocytic leukaemia (CMML) in patients with NF-1 (relative risk 221; 95% CI 71-514), 12 cases of acute lymphoblastic leukaemia (ALL) (relative risk 5.4; 95% CI 2.8-9.4) and five cases of NHL (relative risk 10.0; 95% CI 3.3-23.4). Marrow cytogenetics could be reviewed for seven patients. Specific abnormalities found were monosomy 21 in a child with CMML and 7p+, 17p- in a child with ALL. No abnormalities were reported of 17q, which includes the NF1 gene. CMML occurred predominantly in boys, who also had a family history of NF-1. ALL and NHL were more often found in children with no previous family history.

Adolescent↗

International incidence of childhood brain and spinal tumours.

BACKGROUND: Intracranial and spinal cord tumours are the second most frequent type of childhood cancer after leukaemia, accounting for around 20% of cases in many regions of the world, yet there have been few studies of their incidence by histological type and subsite. METHODS: Age-specific and age-adjusted incidence rates were calculated from data in the study, 'International Incidence of Childhood Cancer', co-ordinated by the International Agency for Research on Cancer. RESULTS: The highest age-adjusted incidence, 31.4 per million, was observed in the Nordic countries, and rates between 24 and 27 per million were found in most other predominantly white Caucasian populations. In the US, black children had a significantly lower incidence (21.7) than whites (26.4). Lower rates were seen in South America, Africa and Asia, the lowest being those for Chinese populations, and for blacks in Africa, both below 15 per million. Among white populations, astrocytomas were the commonest histological type, often with an incidence of at least 10 per million, followed by medulloblastomas, 5-6 per million, and ependymomas, 2-4 per million. In other regions with lower incidence rates, these three types accounted for similar proportions of the total. Black children in the US had a higher incidence of craniopharyngiomas than whites and there was an unusually high incidence of pineal tumours in Japan, 0.9 per million compared with 0.3-0.4 in many other countries. CONCLUSIONS: The low recorded total incidence in developing countries may be partly due to underascertainment. Differences in total incidence or in relative frequencies of particular histological types between Western countries and Japan and between ethnic groups in the US suggest a substantial contribution of genetic predisposition in their aetiology.

Age Distribution↗

International variations in the incidence of childhood soft-tissue sarcomas.

In the study of international childhood cancer incidence coordinated by the International Agency for Research on cancer, soft-tissue sarcomas comprised between 4 and 8% of all cancers between 0 and 14 years of age. Among predominantly white populations, the age-standardised annual incidence rate (ASR) for all soft-tissue sarcomas was between 5 and 9 per million. The most common histological subtypes were rhabdomyosarcoma (ASR 4-7 per million) and fibrosarcoma, including other malignant fibromatous tumours (ASR 1-2 per million). In the United States the incidence of rhabdomyosarcoma for black girls was only half that for white girls, while the rates for boys were similar in the two ethnic groups; fibrosarcoma had a higher incidence among black people than white people for both sexes. Throughout most of Asia, soft-tissue sarcomas almost invariably had a total ASR below 6 per million, rhabdomyosarcoma and fibrosarcoma again being the most common histological types. In Africa, incidence rates could not generally be calculated, but there were substantial numbers of registrations for rhabdomyosarcoma and fibrosarcoma. The majority of cases of Kaposi's sarcoma were in African children, and in eastern and southern Africa where Kaposi's sarcoma is endemic among adult men it comprised 25-50% of soft-tissue sarcomas and 2-10% of all childhood cancers; the sex ratio (M/F) was 2.2:1, in contrast to the overwhelming male predominance among adults. Variations between ethnic groups in the incidence of rhabdomyosarcoma and fibrosarcoma, together with their occurrence in a number of heritable syndromes, suggest that genetic predisposition is important in their aetiology. There was little evidence for an environmental aetiology in rhabdomyosarcoma and fibrosarcoma but an infectious agent may be responsible for childhood Kaposi's sarcoma.

Adolescent↗

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.

Adolescent↗

Childhood cancer.

Trends in incidence for the main types of childhood cancer are analysed using data from the British National Registry of Childhood Tumours; published reports from other registries are summarized. For registries included in Cancer Incidence in Five Continents, changes in rates reported in successive volumes are calculated and a number of problems in the interpretation of these results are identified. There is some limited evidence for a small increase in the incidence of ALL and more evidence for an increase in brain tumours, although the latter may be at least partly due to changes in diagnostic practice. There is limited, but good, evidence for an increase in the incidence of neuroblastoma. With two exceptions, there is no good evidence for any large increase in incidence for any form of childhood cancer in Britain or other countries over the past 20-30 years. The first exception is Kaposi's sarcoma in Uganda, the cumulative incidence in childhood increasing from fewer than 40 per million in 1968-1982 to more than 500 per million in 1989-1991. The second exception is thyroid carcinoma in Belarus, where the reported incidence probably represents about a 50-fold increase--to about 80 per million per year. This may, however, be at least partly a consequence of improved ascertainment following a screening programme. The results presented here suggest that no widespread important risk factor has been introduced over the past 30 years--unless some other similar factor has been simultaneously removed. Analyses of trends in mortality for childhood cancers are now mainly useful as a means of showing the impact of improved treatment methods.

Adolescent↗

International variations in the incidence of childhood carcinomas.

In the study of international childhood cancer incidence coordinated by the International Agency for Research on Cancer, carcinomas were generally rare, with an annual incidence for most sites nearly always under 1/1 million. Liver carcinoma was most common in parts of East Asia, Oceania, and Africa, where it is also common among adults and hepatitis B infection is widespread. Adrenocortical carcinoma had an incidence in Sao Paulo, Brazil, of 1.5/1 million, more than three times the rate in most other registries, indicating the presence of either a specific environmental risk factor acting before or around the time of birth or a concentration of genetically susceptible persons. Thyroid carcinoma seldom had a recorded incidence of more than 1/1 million, and variations in recorded rates may reflect differences in frequency of diagnosis rather than variations in risk. In East Asian populations, who have the highest incidence of nasopharyngeal carcinoma among adults, the childhood incidence of this cancer was moderately elevated. By far the highest incidence in children was found in North Africa, a region of intermediate risk for adults. In the United States the incidence among Black children was nine times that among Whites. Genetic and environmental factors may both be involved in the striking ethnic and geographical distribution. Oral carcinoma in childhood had a high relative frequency in Bangladesh. In the United States, the incidence among Black children was three times that among Whites. Skin carcinoma had an exceptionally high frequency in Tunisia, associated with the unusually high prevalence of xeroderma pigmentosum.(ABSTRACT TRUNCATED AT 250 WORDS)

Adrenal Cortex Neoplasms↗

International variations in the incidence of childhood bone tumours.

Bone cancers comprise about 5% of childhood neoplasms. Osteosarcoma, the most common sub-type, shows a somewhat irregular geographic pattern of incidence, with low rates in some Asian (Indian, Japanese, Chinese) and Latin American populations. Incidence is similar in the sexes and rises steeply with age, accompanied by an increasing proportion of tumours localized in the long bones of the legs. Rates in the USA are higher in blacks than in whites, as a result of a higher incidence at ages 10 to 14 and of tumours of the leg bones. The descriptive epidemiology is consistent with early observations linking risk to the amount of bone growth. Ewing's sarcoma is rare in black populations (USA and Africa) and in eastern Asia. Compared with osteosarcoma, a lower percentage of tumours is localized to the long bones, and incidence rises less steeply with age and is accompanied by an increasing proportion of pelvic tumours. Chondrosarcoma is a rare cancer in children (less than 5% of bone cancers), with an age distribution similar to that of osteosarcoma and a sub-site distribution resembling that of Ewing's sarcoma. Little is known of the aetiology of these tumours; there is clearly a strong genetic predisposition in Ewing's sarcoma but, although the proportion of osteosarcoma cases of genetic origin seems to be small, environmental determinants so far suspected can account for only a small fraction of the total cases.

Adolescent↗

Cancer in Cumbria and in the vicinity of the Sellafield nuclear installation, 1963-90.

OBJECTIVE: To reappraise the epidemiological findings reported by the Black Advisory Group concerning a possible excess of malignant disease, particularly of childhood acute lymphoid leukaemia and non-Hodgkin lymphomas, in the vicinity of the Sellafield nuclear installation, and to determine whether any excess of malignant disease had occurred among people aged 0-24 years in the area in the years after the Black report--that is, from 1984 to 1990. DESIGN: Calculation of incidence of cancer using data from population based cancer registries and special surveys. SETTING: England and Wales; county of Cumbria; county districts Allerdale and Copeland within Cumbria; Seascale ward within Copeland. SUBJECTS: All residents under the age of 75 years in the above areas, but with particular reference to those aged 0-24 years. MAIN OUTCOME MEASURES: Numbers of cases and incidence particularly of lymphoid leukaemia and non-Hodgkin lymphomas in those aged 0-24 years, but including other cancers and age groups. RESULTS: Previous reports of an increased incidence of cancer, especially of leukaemia, among those aged 0-24 years in Seascale during the period up to and including 1983 are confirmed. During 1984-90 there was an excess of total cancer among those aged 0-24 years. This was based on four cases including two cases of non-Hodgkin lymphoma but none of leukaemia. There was an increased, but nonsignificant, incidence of other cancers, based on two cases (one pinealoma and one Hodgkin's disease) occurring among those aged 15-24 years during 1984-90. This was not observed in the younger age group or in previous years. For the immediately surrounding area--that is, the county districts of Allerdale and Copeland excluding Seascale and in the remainder of Cumbria--there was no evidence of an increased incidence of cancer among those aged 0-24 years in either period. CONCLUSIONS: During 1963-83 and 1984-90 the incidence of malignant disease, particularly lymphoid leukaemia and non-Hodgkin lymphomas, in young people aged 0-24 in Seascale was higher than would be expected on the basis of either national rates or those for the surrounding areas. Although this increased risk is unlikely to be due to chance, the reasons for it are still unknown.

Adolescent↗

Chemotherapy in the management of infantile fibrosarcoma.

Infantile fibrosarcoma (IF) has traditionally been treated with surgery, which may have considerable morbidity. Chemotherapy has been suggested in order to reduce the need for extensive surgery. Nine children with histologically confirmed IF who received chemotherapy are described. Six children were treated with chemotherapy initially, two following conservative surgery, and one following recurrence after surgery. All received vincristine (V) and actinomycin D (A), and six received additional drugs including ifosfamide (I), cyclophosphamide (C), adriamycin (Ad), etoposide (E), and cisplatinum (CDDP). Objective responses were achieved in eight: three responded completely (CR), two responded partially (PR), which allowed conservative surgery, one had stable disease, one had an initial PR, but subsequently had tumour recurrence 1 month after cessation of treatment, necessitating further surgery and chemotherapy, and one had an initial PR but died following local and regional metastases. One child had no response to chemotherapy but is alive with stable residual disease. Thus, five of nine children achieved a CR--three with chemotherapy alone. With the inclusion of chemotherapy as part of their treatment, five children, for whom curative surgery may have resulted in amputation, remain alive with limbs intact. Chemotherapy including V and A should be given to infants with fibrosarcoma in whom curative surgery would be mutilating.

Antineoplastic Combined Chemotherapy Protocols↗