Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023418 (leukemia)
 93,477 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The frequency of different malignant cutaneous tumors (MCTs), primary and metastatic, in children is not known. We reviewed all MCTs, primary and metastatic, seen during a 20-year period in a large general pediatric hospital. Fifty-three MCTs, 36 primary and 17 metastatic, were diagnosed in 36,207 pediatric dermatology patients. The incidence was 1.4 per 1000 patients. The relative frequency of occurrence of the different tumors was as follows: rhabdomyosarcoma, 25%; lymphomas, 19%; basal cell carcinoma, 13%; leukemia, 13%; neuroblastoma, 10%; malignant melanoma, 6%; squamous cell carcinoma, 6%; unclassified sarcomas, 4%; epithelioid schwannoma, 2%; ependymoma, 2%. The mean follow-up was 3 years; 48% died, 27% were lost to follow-up, and 25% are under control. We conclude that primary and metastatic MCTs in children are rare. Their types differ from MCTs in an older age population. MCTs in children are associated with a high mortality rate, often related to late recognition.
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PMID:Malignant cutaneous tumors in children. Twenty years of experience at a large pediatric hospital. 828 84

Fifteen children ranging in age from 4 to 18 years with leptomeningeal metastases were evaluated for extent of tumor and treated with radiotherapy or chemotherapy. Histologic diagnosis included: acute lymphoblastic leukemia (4); anaplastic astrocytoma (1); ependymoma (2); nonseminomatous germ cell tumor (1); non-Hodgkin's lymphoma (1); pineoblastoma (1); and primitive neuroectodermal tumor not otherwise specified (2). Pretreatment imaging studies demonstrated recurrent intracranial parenchymal disease in nine, spinal disease in four, and abnormal radioisotope ventriculography in six. Systemic disease was seen in four children (3 leukemia; 1 non-Hodgkin's lymphoma). All children were treated with systemic chemotherapy and intraventricular chemotherapy. Nine children received radiotherapy to bulky or symptomatic leptomeningeal disease. Median survival was 6 months (range, 4-18 months). Children with hematologic malignancies had superior outcomes compared to children with solid tumors. Three children with leukemic or lymphomatous meningitis are alive and disease free whereas all children with carcinomatous meningitis died. In conclusion, leptomeningeal metastases in children portends a limited survival, and therapies at this time remain palliative except in children with hematologic malignancies.
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PMID:Pediatric leptomeningeal metastases: outcome following combined therapy. 901 Jul 96

In the framework of the ITACARE project, a cooperative investigation conducted on the data from the Italian population-based cancer registries, survival of patients with childhood malignant neoplasms was studied. The study included 1,768 cases diagnosed at age 0-14 plus 29 osteosarcoma cases diagnosed at age 15-19. Cases were collected over the period 1978-1989, or more limited periods for some participating registries. A total of 1,138 cases were from the Childhood Cancer Registry of Piedmont and 659 from the registries operating in the provinces of Varese, Parma, Modena, Forli and Ravenna, Florence, Latina, Ragusa and in the cities of Genova and Torino (the last contributed only for bone neoplasm diagnosed at age 15-19). Overall 5-year survival was 54% for malignancies diagnosed in 1978-1981, 60% for the period 1982-1985; and 69% for the period 1986-1989. The range among registries of 5-year survival for cases diagnosed in 1986-1989 was 55-78%. Most diagnostic categories presented an improved prognosis for the cases diagnosed more recently. For cases diagnosed in 1986-1989, 5-year survival was: 74% for acute lymphatic leukaemia, 40% for acute non-lymphatic leukaemia, 65% for central nervous system neoplasms (76% for astrocytoma, 75% for ependymoma and 85% for medulloblastoma), 66% for osteosarcoma, 55% for Ewing's sarcoma, 87% for Hodgkin's disease, 64% for non-Hodgkin's lymphoma, 74% for rhabdomyosarcoma, 64% for neuroblastoma, 78% for nephroblastoma and 100% for retinoblastoma. Italian survival was similar to that observed in other population-based surveys in the UK and USA.
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PMID:Survival of childhood cancer patients in Italy, 1978-1989. ITACARE Working Group. 915 68

Graft-versus-leukemia effect is an immune-mediated antitumor phenomenon associated with allogenic bone marrow transplants (BMTs) for hematological malignancies, and recent findings have indicated that a similar effect could occur in some solid tumors such as breast cancers. The authors report on a 42-year-old man with a recurrent ependymoma who received an allogenic BMT for therapy-related leukemia. After transplantation, the patient developed chronic graft-versus-host disease, which was controlled with steroid agents. Interestingly, the recurrent ependymoma regressed steadily over the next 21 months posttransplant, until the tumor became almost undetectable on magnetic resonance images. This case indicates that the graft-versus-tumor effect, mediated by cytotoxic T cells, may be able to target intraparenchymal neuroepithelial tumors, despite the brain's generally recognized status as an immunoprivileged organ.
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PMID:A graft-versus-tumor effect in a patient with ependymoma who received an allogenic bone marrow transplant for therapy-related leukemia. Case report. 1218 80

Idarubicin (IDA), the 4-demethoxy analog of daunomycin, has had significant cytotoxicity in many malignancies. In previous reports, the alcohol metabolite of IDA, 4-demethoxydaunorubicinol (idarubicinol, or IDOL), had cytotoxic activity and the ability to penetrate the blood-brain barrier. For this reason, the Pediatric Oncology Group conducted a Phase 2 trial of IDA for children with recurrent or progressive brain tumors. Ninety-one eligible children were entered on this study, with ages ranging from 3 months to 19 years. Patients were stratified by tumor types into 6 categories: stratum 1, low-grade astrocytoma; stratum 2, malignant glioma (glioblastoma multiforme and anaplastic astrocytoma); stratum 3, medulloblastoma; stratum 4, brainstem glioma; stratum 5, ependymoma; and stratum 6, miscellaneous malignant tumors not included in the previous diagnoses. IDA(18 mg/m2) was infused over 4 h and followed by granulocyte colony-stimulating factor (G-CSF) beginning day 5 after infusion of IDA. G-CSF was continued until blood cell count recovery. Cycles were repeated at approximately 21-day intervals until patients developed progressive disease or had completed 6 cycles with stable or improved disease. Pharmacokinetic plasma and cerebrospinal fluid (CSF) samples were collected from a subset of these patients. Response was poor in all strata. Most patients developed progressive disease; however, in 21 patients with medulloblastoma there was 1 partial response, and 6 patients had stable disease (SD) that in 4 patients lasted more than 20 weeks. Grades 3/4 hematopoietic toxicities were the most common toxic events, and 14 infection-related events resulted in hospitalization of patients. Only 1 patient developed reduced cardiac function. The systemic clearance data for IDA and IDOL were nearly identical to those published on patients with leukemia, and the plasma elimination of the IDOL metabolite was substantially longer than that of the parent drug IDA. The peak CSF:plasma ratios of IDA and IDOL were very low. The overall response rates to IDA were disappointing despite periods of prolonged SD in nearly a fourth of the patients. We conclude from this data and from that in nonhuman primates that it is unlikely that IDA, daunomycin, or other related anthracyclines will be useful for treating primary CNS tumors.
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PMID:Phase 2 study of idarubicin in pediatric brain tumors: Pediatric Oncology Group study POG 9237. 1456 63

Childhood leukaemia has a potential infectious aetiology whilst infections may also be linked to paediatric central nervous system (CNS) tumours. Using data from 29 countries we investigated the correlation between international incidence rates of childhood leukaemia and CNS tumours, focusing on acute lymphoblastic leukaemia (ALL), astrocytoma and ependymoma-subtypes that are hypothesised to have an infectious aetiology. Relationships between incidence rates and national demographic factors were also examined using Pearson's correlation coefficient to quantify associations. Comparing two diagnostic categories of leukaemia with four groups of CNS tumours, a highly significant positive correlation was found between ALL and astrocytoma (r = 0.57, P = 0.002). Higher rates of ALL and CNS tumours were associated with increased affluence, with the strongest correlation for Gross Domestic Product per capita and CNS tumours (r = 0.70, P < 0.001). National incidence rates of childhood ALL and astrocytomas were highly correlated and this may reflect a common environmental cause whose origin may be infectious in nature. International incidence of ALL and CNS tumours were also correlated with economic related factors. Variation in levels of ascertainment may partially explain this, although childhood environmental exposures related to infections will also be affected by levels of affluence.
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PMID:Childhood leukaemias and CNS tumours: correlation of international incidence rates. 1641 49

The main properties of stem cells include long-term self-renewal and the capacity to give rise to one or more types of differentiated progeny. Recently, much evidence was provided that leukemia and tumor maintenance and growth are sustained by a small proportion of cells exhibiting stem cell properties. In neural tumors, stem cells have been detected in glioblastoma, medulloblastoma and ependymoma. These observations imply that normal stem cells could be the origin of cancer stem cells; alternatively, a more differentiated progeny may revert to a "stem-like" status, and give rise to cancer stem cells. In adult brain residual stem cells are located in the hippocampus, the subventricular zone and possibly the cerebellum. However, evidence for the ability of more differentiated progeny (astroglia, oligodendroglia) to convert into "stem cells" in vitro has also been provided, thus greatly expanding the potential target of oncogenic mutations. In the framework of the cancer stem cell hypothesis, genes originally identified as important for normal neural stem cells may be essential to support cancer stem cells as well. Stem cell genes act in several ways: they stimulate stem cell self-replication, inhibit differentiation, control excessive replication that might lead to "exhaustion" of the stem cell pool. Mutations in man and mouse, in spontaneous or experimental brain tumors, often target stem cell genes or genes lying in their functional pathway, the main examples being the Sonic hedgehog and the Wnt pathways. Interestingly, several stem cell genes are often overexpressed in brain tumors, even if they are not mutated. This suggests that these genes may be important for the generation of cancer stem cells from more differentiated precursors, or for cancer stem cell maintenance. Cancer stem cells partially differentiate in vivo, and in vitro they also give rise to seemingly normal differentiated progeny, like normal stem cells: thus, their main defect, leading to cancer, may lie in the unbalance between self-replication and terminal differentiation of this minority cell population. Knowledge of extrinsic diffusible factors affecting the activity of stem cell genes may help identifying tools for inducing cancer stem cell differentiation, which might be of use in therapy.
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PMID:Cancer stem cells and "stemness" genes in neuro-oncology. 1714 9

To study the occurrence of leukemia as a second malignancy following various primary solid and hematological malignancies. Total 11 cases of leukemia presenting as a second malignancy were studied over a period of 15 years from 1990 to 2005. The primary malignancies included carcinoma breast (4), multiple myeloma (3) and one each of Hodgkin's lymphoma, mediastinal germ cell tumor, papillary carcinoma thyroid and myxopapillary ependymoma. Ten patients had received chemotherapy with combination radiotherapy in six patients. The commonest type of leukemia was AML-M2. The cyogenetic test results were available in three cases. The secondary leukemia showed aggressive behaviour and all patients on follow-up died within a period of one month. The risk benefit ratio of chemotherapy and radiotherapy should be considered before starting the patients on treatment. A high degree of suspicion and follow up with hematological parameters is required for therapy related complications.
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PMID:Leukemia as a second malignancy. 1788 71

This paper reports the latest survival data for French childhood cancer patients at the national level. Data from the two French National Registries of Childhood Cancer (Haematopoietic Malignancies and Solid Tumours) were used to describe survival outcomes for 15,479 children diagnosed with cancer between 2000 and 2008 in mainland France. The overall survival was 91.7% at 1 year, 86.9% at 2 years and 81.6% at 5 years. Relative survival did not differ from overall survival even for infants. Survival was lower among infants for lymphoblastic leukaemia and astrocytoma, but higher for neuroblastoma. For all cancers considered together, 5-year survival increased from 79.5% in the first (2000-2002) diagnostic period to 83.2% in the last (2006-2008) period. The improvement was significant for leukaemia, both myeloid and lymphoid, central nervous system tumours (ependymoma) and neuroblastoma. The results remained valid in the multivariate analysis, and, for all cancers combined, the risk of death decreased by 20% between 2000-2002 and 2006-2008. The figures are consistent with various international estimates and are the result of progress in treatment regimens and collaborative clinical trials. The challenge for the French registries is now to study the long-term follow-up of survivors to estimate the incidence of long-term morbidities and adverse effects of treatments.
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PMID:Childhood cancer survival in France, 2000-2008. 2501 Aug 37

Exposure to benzene increases the risk for acute myeloid leukemia and possibly other types of cancer in adults. For children, only limited evidence about benzene and cancer exists. A few studies have indicated that benzene may increase risk for some subtypes of childhood cancer but not for others. We aimed to investigate if outdoor levels of benzene at the residence increase the risk for subtypes of leukemia, lymphoma and CNS tumor in children. We identified 1,989 children diagnosed with leukemia, lymphoma or CNS tumor during 1968-1991 in the Danish Cancer Registry and randomly selected 5,506 control children from the Danish population, matched on sex, age and calendar time. We traced residential history of all children from 9 months before birth to time of diagnosis, calculated outdoor benzene concentration at all addresses and summarized cumulative exposure over fetal and childhood periods separately. We used conditional logistic regression for the statistical analyses. Benzene exposure during childhood above the 90th percentile was associated with relative risks for acute lymphocytic leukemia (ALL) and acute myeloid leukemia (AML) of 1.0 (95% confidence intervals (CI): 0.6-1.7) and 1.9 (95% CI: 0.3-11.1), respectively, when compared with exposure levels below the median. For CNS tumors, there was a tendency of lower risk for ependymoma and higher risk for medulloblastoma in association with higher exposure. In conclusion, benzene was associated with higher risk for childhood AML, but not ALL, which is consistent with the few previous studies.
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PMID:Ambient benzene at the residence and risk for subtypes of childhood leukemia, lymphoma and CNS tumor. 2963 47


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