Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027819 (neuroblastoma)
 27,800 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The International Agency for Research on Cancer has coordinated a worldwide study of childhood cancer incidence, with data from over 50 countries. We present here the results for neuroblastoma. In predominantly white Caucasian populations the age-standardized rate was 7-12 per million, and 6-10% of all childhood cancers were neuroblastomas. Rates were highest in the first year of life (25-50 per million, 30% of total neuroblastoma incidence), and decreased with age to 15-20 per million (50% of the total) at age 1-4, 2-4 per million (15%) at 5-9 and 1-1.5 per million (5%) at 10-14. In the United States, black children had an incidence of 8.5 per million compared with 11.5 among Whites; Blacks tended to be older than Whites at diagnosis. The highest rate in Africa was in Bulawayo, Zimbabwe (8.0 per million) and the lowest in West Nile, Uganda, with no cases registered. Incidence in Israel was similar to that in many white populations, with Jews having a particularly high rate. In other parts of West Asia neuroblastoma had a low relative frequency, suggesting that incidence is low. Rates were also low throughout much of southern and eastern Asia, including India and China. Incidence in Japan was somewhat higher, though less than in Western countries, with the deficit most pronounced in the first year of life; these data relate to the period before mass screening of infants for neuroblastoma in the regions concerned. Incidence was generally higher in regions and among ethnic groups enjoying a higher standard of living, though previous studies within single countries had suggested that neuroblastoma is more common among less affluent groups. Blacks in Africa and the United States may have a weaker genetic predisposition to neuroblastoma, but some of the deficit in many developing countries is likely to be due to under-diagnosis.
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PMID:International variations in the incidence of neuroblastoma. 851 61

This paper reviews current approaches to the use of ifosfamide/mesna alone or in combination with other agents or modalities in the treatment of pediatric malignancies. Included are data from current or recently completed studies conducted by major pediatric oncology cooperative groups and large individual oncology centers for patients with newly diagnosed or recurrent tumors. Sarcomas, neuroblastoma, lymphomas, recurrent solid tumors, brain tumors, and acute lymphoblastic leukemia are discussed. Randomized trials to determine the relative efficacy of ifosfamide and cyclophosphamide in various childhood malignancies are under way. The long-term consequences of ifosfamide in survivors of childhood cancer, in terms of development of bladder cancer or other malignancies thought to be associated with alkylating agents, are not known, and will only be determined through follow-up studies of adult survivors. Ifosfamide's future role in pediatric oncology will depend on evaluation of its therapeutic benefits against long-term toxicity.
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PMID:Current studies of ifosfamide for pediatric solid tumors and leukemia in the United States. 148 74

A case-control study was conducted 1) to determine whether maternal prenatal drug consumption increases the risk of neuroblastoma in the child and, if so, 2) to see if the size of the risk depends on whether the case is inherited or sporadic. Mothers of children with newly diagnosed neuroblastoma (n = 101) were compared with mothers of children newly diagnosed with other forms of childhood cancer (n = 690). Cases and controls were selected from the population of childhood cancer patients at St. Jude Children's Research Hospital, Memphis, Tennessee, between 1979 and 1986. The patients' mothers were interviewed to ascertain their prenatal medication, alcohol, and tobacco consumption patterns. Unconditional logistic regression models were used to adjust for maternal age at birth, patient age at diagnosis, race, social class, exposure to x-ray, miscarriage, and other confounding variables. Adjusted odds ratios were estimated for the total sample and for subgroups that had a higher probability of containing inherited cases. Drugs associated with neuroblastoma case status include diuretics for hypertension (odds ratio (OR) = 4.1, 95 percent confidence interval (CI) 1.0-16.9), tranquilizers (OR = 2.1, 95 percent CI 1.1-4.3), nonprescription pain relievers (OR = 1.9, 95 percent CI 1.1-3.1), and cigarettes (OR = 1.9, 95 percent CI 1.1-3.2). The odds ratios for maternal prenatal drug consumption for the group with inherited cases and the total sample were approximately the same. This equality is inconsistent with predictors based on Knudson's two-stage model of carcinogenesis.
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PMID:Influence of the mother's prenatal drug consumption on risk of neuroblastoma in the child. 151 82

To evaluate the significance of the peripheral lymphocyte count in the prognostication of childhood cancer, 173 children with cancer (neuroblastoma, non-Hodgkin's lymphoma, malignant lymphogranuloma, nephroblastoma, Ewing's sarcoma, and rhabdomyosarcoma) were studied. All patients with the above-mentioned diagnoses admitted for the first time between 1985 and 1987 without prior treatment and acute infection were eligible for the study. Elevated peripheral lymphocyte count seems to be an independent indicator of survival from neuroblastoma but not from other tumors.
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PMID:Lymphocyte count as a prognostic factor in childhood cancer. 152 95

Cultured human neuroblastoma cells can be classified morphologically into 3 types: neuroblastic (N), intermediate (I) and substrate adherent (S). Neuroblastoma cells of all types were found to attach and display distinct morphological characteristics on fibronectin, with S-type cells attaching better than N-type cells. Studies of the expression of integrin fibronectin receptors (alpha 3 beta 1, alpha 4 beta 1, alpha 5 beta 1 and alpha V beta 1) were carried out using a total of 26 morphologically distinct cell lines and their subpopulations. Fluorescence-activated cell sorting (FACS) analysis and immunoprecipitation revealed that all S-type cells expressed abundant alpha 5 beta 1, while N-type cells barely expressed this molecule. Although alpha 3 beta 1 expression of S-type cells was also higher than that of N-type cells, some N-type cells had significantly increased levels of this molecule. alpha 4 beta 1 was found to be randomly expressed. All cell lines tested expressed alpha V beta 1. Human neuroblastoma cells, the majority of which are N-type cells with very low alpha 5 beta 1 expression, are also contrasted with other childhood cancer cells (rhabdomyosarcoma, Ewing's sarcoma, and glioma), all of which expressed high levels of alpha 5 beta 1. The characteristic expression of integrin fibronectin receptors may account for the clinically unique tumor behavior, and the immunohistochemical staining for integrins may become a useful alternative to conventional histology in differential diagnosis and a marker for prognosis in neuroblastoma.
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PMID:Unique expression of integrin fibronectin receptors in human neuroblastoma cell lines. 153 85

Incidence rates for pediatric (ages, 0 to 14 years) cancer in the state of Florida were produced for the period 1981 to 1986 and compared with national data to investigate variations in pediatric cancer incidence. Overall, Florida had an incidence rate of 12.77 per 100,000; this was not significantly higher than expected based on national rates (standardized incidence ratio, 1.0; 95% confidence interval, 0.9 to 1.0). Compared with national rates, whites residing in Florida had an increased rate of acute lymphocytic leukemia and decreased rate for soft tissue sarcomas (other than rhabdomyosarcoma), "other leukemias," and "other" cancers. Nonwhites residing in Florida had increased rates for soft tissue sarcomas (other than rhabdomyosarcoma) and decreased rates of "other" cancers. Boys in Florida had increased rates for retinoblastoma and acute lymphocytic leukemia and decreased rates for "other leukemias" and "other" cancers. Rates for girls were decreased for neuroblastoma and "other leukemias."
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PMID:The incidence of pediatric cancer in Florida, 1981 to 1986. 154 26

The sequential outcome was evaluated for all childhood cancers in which the Pediatric Oncology Group has conducted a series of clinical trials, with constant eligibility, on patients with newly diagnosed cancer. The analysis was applied to more than 7000 patients with cancer diagnosed between 1976 and 1989. These include acute leukemia (4 subgroups), non-Hodgkins lymphoma (4 subgroups), osteogenic sarcoma, and advanced neuroblastoma. In 8 of these 10 disease areas, significant improvement in outcome has occurred. In rare diseases such as pediatric cancer, collaborative studies may be the only way to conduct therapeutic trials of sufficient statistical power. A cooperative group has distinct advantages over a series of ad hoc collaborative studies in that it can maintain a unified data base, study its history with minimal confounding effects of changing institutional participants, and develop long-term research relationships among its participants.
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PMID:Progress against childhood cancer: the Pediatric Oncology Group experience. 155 37

The purpose of this study is to describe the incidence and survival of childhood cancer in the West Midlands for the period 1980-1984. Proportional breakdown by Asian subgroup is also considered. A total of 587 patients were registered, 49 of them of Asian origin. Breakdown to Asian versus non-Asian subgroups by diagnosis revealed comparatively high rates for Hodgkin's disease, retinoblastoma and neuroblastoma in the Asian patients. However, a deficit of cases was seen for CNS tumours. Comparison of overall age-standardized rates (ASR) for all cancers revealed a substantially lower value compared to that reported for the USA white population but a similar value to the USA black and UK white populations. Diagnostic breakdown revealed that the major difference between the West Midlands Regional Children's Tumour Research Group (WMRCTRG) and the USA white ASR was in the leukaemia and lymphoma group. Overall survival for the series was 56% at 5 years. The poorest prognosis was found in acute myeloid leukaemia, with only 23% of patients surviving at 5 years, against 62% in acute lymphoblastic leukaemia. CNS tumours also had a poor outcome, with an overall survival rate of 47%, although certain individual diagnoses were more favourable. We observed a 100% survival rate in Hodgkin's disease up to 5 years from diagnosis, and both Wilms' tumour and retinoblastoma had 90% survival rates.
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PMID:Childhood cancer in the West Midlands: incidence and survival, 1980-1984, in a multi-ethnic population. 158 36

Immunological evaluations were performed on 14 pediatric cancer patients who received human recombinant interleukin-2 (rIL-2) as a bolus intravenous infusion every 8 h for 5 consecutive days in a phase I trial. Three-to-four patients were treated at dose levels of 10, 30, 60, and 100 x 10(3) Cetus U/kg. Six of the patients had stage D neuroblastoma; the remainder had other solid tumors or leukemias. Infusion of rIL-2 was associated with a rapid margination of IL-2-responsive cells followed by demargination and heightened proliferative and cytotoxic activity after therapy was completed. The predominant phenotypic change in circulating peripheral blood mononuclear cells (PBMC) was an increase in CD2 expression by CD56+ natural killer (NK) cells. Appearance of CD2+ CD56+ cells in the circulation correlated with increased lymphokine-activated killer (LAK) cell activity as defined by the ability to kill NK-resistant Daudi tumor cells in vitro. Sustained LAK activity appeared to be dependent on the bioavailability of rIL-2 in vivo as well as in vitro. After rIL-2 therapy, PBMC that were highly responsive to rIL-2 (activated and "poised" LAK cells) persisted for at least 72 h. In the patients tested, increased lysis of autologous and/or allogeneic, histologically similar tumor cell lines was also observed after therapy. The immunoenhancing effects of rIL-2 occurred even at the lower doses used in this study. However, an objective tumor response was not observed in any of the patients.
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PMID:Immunological evaluation of pediatric cancer patients receiving recombinant interleukin-2 in a phase I trial. 159 13

Between 1987 and 1991, 248 long-term survivors of childhood cancer have been studied at the Oncology Unit of the Children's Hospital of Buenos Aires. The tumors were diagnosed between 1965 and 1986 as, retinoblastoma: 65, lymphoma: 57, nephroblastoma: 36, neuroblastoma: 25, germ-cell tumors: 18, sarcoma: 19, bone tumors: 7, lymphoepithelioma of cavum: 4, histiocytosis X: 9, others: 8. The treatment consisted of, surgery: 25, surgery+chemotherapy: 40, surgery+radiotherapy: 8, chemotherapy: 23, chemotherapy+radiotherapy: 42, and surgery+chemotherapy+radiotherapy: 110. There are alive without evidence of cancer disease 234 survivors between 5 and 25 years after diagnosis. Severe organic disabilities were observed in 181 survivors and moderate in 142. Thirteen patients died because of second malignant neoplasia and 1 patient with lung metastasis 9 years after diagnosis of nephroblastoma. In 180 survivors the data of instruction was available. Seventy participate in sports and 13 in artistic activities. Eight survivors are university graduates and 24 are employees. Five young women were mothers and one young man was a father. The meaning of concept of "cure" is discussed from the point of view of the physicians and the survivors. In order to detect deleterious late effects of cancer and their treatment the follow-up must be continuous.
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PMID:[The concept of cure in children with cancer]. 182 19


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