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)

Consistent cytogenetic abnormalities have been described in many pediatric solid tumors, including Ewing's sarcoma, Wilms' tumor, and neuroblastoma. Similar analysis of pediatric central nervous system (CNS) tumors has been hampered by technical problems. We report chromosome results from 39 pediatric CNS tumors. Abnormalities of chromosome 17 were noted in 3 of 11 primitive neuroectodermal tumors (including i(17q) in 2 tumors), confirming data observed by other investigators. Cells from 2 of 11 primitive neuroectodermal tumors (PNET) exhibited loss or structural abnormalities involving chromosome 11. Loss or distal deletion of chromosome 7q was noted in cells from two PNETs. Because other investigators have shown loss of heterozygosity on 17p in about one-third of PNET, we propose that chromosome regions 7q and 11 are areas worthy of further study in pediatric PNET. Numerical abnormalities were noted in 6 of 21 astrocytomas. Hyperdiploidy was demonstrated in 1 of 4 pilocytic astrocytomas and pseudopolyploidy was demonstrated in 4 of 13 anaplastic astrocytomas. Structural chromosome abnormalities (translocations, deletions) were noted in 4 of 13 anaplastic astrocytomas. Complex structural anomalies were observed in one craniopharyngioma. A rhabdoid tumor of the brain exhibited multiple complex structural rearrangements but did not exhibit the monosomy 22 observed in some rhabdoid tumors. Hypodiploidy and loss of chromosome 22 were noted in a clinically aggressive meningioma, corroborating observations by other investigators.
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PMID:Cytogenetic analysis of 39 pediatric central nervous system tumors. 131 29

116 infants under one year of age (14 per 100) with malignancies including 35 neuroblastomas, 23 retinoblastomas, 14 Wilm's tumours, 10 hepatoblastomas, 10 brain tumours, 9 germ cell tumours, 8 histiocytic and 7 soft tissue sarcomas, were treated in the last ten years. Hepatoblastoma is the highest relative incidence tumor in the first year of life and with brain tumours has the worst prognosis (50 and 40 per 100, respectively). The disease-free survival rate is most than 80 per 100 in neuro and nephroblastoma with medical and surgical treatment. Familiar incidence in Wilm's tumor is 42 per 100 and chemotherapy side effects are 23 per 100. Familiar incidence in neuroblastoma is 20 per 100 and chemotherapy side effects 21 per 100. In conclusion, in children with malignancies under one year of age have good prognosis (75 per 100 survival at five years), with lower surgical complications rate. Early diagnosis and response to chemotherapy, the side effects of which are considerable and should be strictly controlled, are key factors in the better prognosis and increased life expectancy in this group of patients.
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PMID:[Neoplasms during the 1st year of life: a study of 116 cases treated during the last 10 years]. 131 38

Levels of serum manganese superoxide dismutase (Mn-SOD) in normal children aged from 1 to 14 years and children with various hematological and malignant diseases were determined by enzyme-linked immunosorbent assay (ELISA). In the normal children, the serum Mn-SOD levels gradually increased in proportion to age. By 8 years of age, the Mn-SOD level was nearly at the adult level. The normal values of serum Mn-SOD (mean +/- SD) of children below 4 and above 8 years old were 48 +/- 10.2 ng/ml and 84 +/- 22.5 ng/ml, respectively. Assuming the upper limit of normal Mn-SOD level in serum to be the mean value +/- 2 SD of children at each age, high serum levels of Mn-SOD were found for 8 of 12 patients with neuroblastoma, three of four patients with Wilms tumor, and four of five patients with acute myeloid leukemia. The patients with neuroblastoma exhibited a transient increase in Mn-SOD following chemotherapy, but after 1 week the levels decreased markedly to the control levels. The changes in serum Mn-SOD levels in the patients with neuroblastoma correlated well with the levels of neuron-specific enolase. Mn-SOD was intensely stained in bone marrow cells of patients whose cancer cells had moved into the bone marrow. High levels of Mn-SOD were also found in cultured human neuroblastoma cells. These data indicate that Mn-SOD is expressed in neuroblastoma cells, may serve as one of the diagnostic and prognostic markers for the neuroblastoma, and may be useful to predict the effectiveness of chemotherapy for neuroblastoma and the recurrence of this disease.
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PMID:High levels of Mn-superoxide dismutase in serum of patients with neuroblastoma and in human neuroblastoma cell lines. 131 10

The majority of the common pediatric cancers are managed on clinical protocols and are treated in pediatric oncology centers. For this reason these neoplasms are being staged by a variety of protocols depending on the protocol study. Examples of the evaluation and currently used staging systems for Wilms' tumor, neuroblastoma, and rhabdomyosarcoma are presented. The goal is to develop a nationally accepted staging system for these common pediatric tumors that is broadly accepted for treatment planning, determining prognosis, and comparing institutional end results. It is obvious that, if a nationally accepted staging system is to evolve, the leadership of the existing national clinic research trials must be part of the process.
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PMID:Staging of pediatric cancers: problems in the development of a national system. 131 8

The results of roentgeno-endovascular intervention in nephroblastoma in 31 child are presented. For chemoembolization, suspension of lipiodol and adriamycin was used, for control of the effectiveness of the intervention--the radiopharmaceutical preparation pyrophosphate. After embolization, the decrease in tumor size was noted. This made easier the performance of nephrectomy, the volume of blood loss reduced. Pathomorphism of neuroblastoma induced by chemoembolization was studied. No complications were noted.
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PMID:[Chemoembolization of blood vessels in the complex treatment of children with nephroblastoma]. 132 80

Carminomycin is an original antitumor antibiotic from the anthracycline group isolated at the Institute of New Antibiotics (USSR) in 1973. Pharmacological investigation of carminomycin revealed its satisfactory absorption from the gastrointestinal tract which proved to be a distinguishing property of the antibiotic as compared to other anthracyclines such as adriamycin and rubomycin. The clinical trials of carminomycin showed that it was mainly active against soft tissue sarcoma and breast cancer, lymphosarcoma, neuroblastoma, Wilms' tumor and Ewing's sarcoma in children, as well as acute leukemia. Various regimens for the antibiotic administration were applied: short-term, single and long-term. Suppression of hemopoiesis was considered as a limiting toxic effect. By the data available carminomycin had lower cardiotoxicity as compared with rubomycin and adriamycin. Development of oral carminomycin is believed promising.
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PMID:[Experience with using carminomycin in oncological clinical practice]. 132 45

P-Glycoprotein (P-gp), the product of the mdr-1 gene, is implicated in the development of chemoresistance in a variety of, mostly adult, cancers. Its role in paediatric tumours, most of which are non-epithelial in origin, has yet to be fully elucidated. A study was undertaken to investigate reactivity of two P-gp monoclonal antibodies (MAbs), JBS-1 and MRK16, recognising cytoplasmic and surface epitopes, respectively, of the P-gp molecule, in a variety of newly diagnosed and relapsed childhood cancers. P-gp was not expressed in any of 36 tumours examined (neuroblastoma 13, nephroblastoma 12, rhabdomyosarcoma 6, lymphoma 3, teratoma 1, Ewings 1), 14 of whom had chemoresistant disease. Reactivity to both MAbs was also investigated in patients with acute leukaemia. Out of 10 diagnostic acute lymphoblastic leukaemia (ALL) samples, a positive reaction with JSB-1 was observed in 1 patient who failed to remit on standard induction therapy and in 3 of 6 patients in ALL relapse, only 1 of whom showed low grade positivity with MRK16. Both MAbs reacted positively in 1 patient with acute non-lymphocytic leukaemia (ANLL) at diagnosis who achieved remission with teniposide and cytosine arabinoside, but relapsed 7 months later and was again positive with both Mabs. JSB-1 also showed varying degrees of positivity in 4 out of 4 other patients in ANLL relapse. It would therefore appear that P-gp is unlikely to mediate chemoresistance in most solid tumours of childhood, but may well play a major role in the development of chemoresistance in acute leukaemia.
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PMID:Reactivity of P-glycoprotein monoclonal antibodies in childhood cancers. 135 71

One hundred twenty-eight palpable and deep-seated fine-needle aspiration biopsies (FNAB) were done on pediatric patients at James Whitcomb Riley Hospital for Children and Indiana University Hospital between 1985 and 1988. During that 4-year period, 71 (56%) benign and 49 (38%) malignant diagnoses were made. Only eight (6%) of the FNAB were considered inadequate. Thirty-nine (80%) of the malignant aspirates were small round blue cell tumors of childhood (SRBCT). The SRBCT consisted of 21 (54%) lymphomas, 7 (18%) Ewing's sarcomas, 3 (8.5%) neuroblastomas, 3 (8.5%) rhabdomyosarcomas, 2 (5.0%) medulloblastomas, 2 (5.0%) Wilms' tumors, 1 (3.0%) retinoblastoma, and 1 (3%) granulocytic sarcoma. Fifteen (38%) of the SRBCT aspirates were obtained to render a primary diagnosis and 24 (62%) documented recurrence. Various combinations of electron microscopy, immunocytochemistry, and other special stains were used to confirm the diagnosis in 11 (28%) cases. These cases consisted of five lymphomas, two rhabdomyosarcomas, two Ewing's sarcomas, one neuroblastoma, and one granulocytic sarcoma. The technique of FNAB is a successful diagnostic tool for documenting primary and recurrent SRBCT in a pediatric population.
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PMID:Fine-needle aspiration biopsy of small round blue cell tumors of childhood. 137 Sep 16

The secretion of insulin-like growth-factor-binding proteins (IGFBPs) and expression of the genes encoding IGFBP-1, IGFBP-2 and IGFBP-3 have been studied in a panel of cell lines derived from breast carcinomas, Wilms' tumour, neuroblastoma, retinoblastoma, colon carcinoma, liver adenocarcinoma, Burkitt's lymphoma and a non-small-cell lung carcinoma. All cell lines, with the exception of the Burkitt's lymphoma cell line, secreted IGFBPs, as detected by affinity labelling. A 34-kDa BP was present in the conditioned media of all IGFBP-secreting cell lines, whereas BPs ranging from 18 kDa to 53 kDa were variably secreted. All IGFBP-secreting cell lines expressed the IGFBP-2 gene as determined by Northern blot analysis. The Wilms' tumour, the neuroblastoma and the retinoblastoma cell line expressed the IGFBP-2 gene only. All other cell lines, with the exception of the Burkitt's lymphoma, expressed the IGFBP-2 gene and, in addition, either the IGFBP-1 gene and/or the IGFBP-3 gene. IGFBP-1 gene expression could be detected by reverse transcriptase polymerase chain reaction only. IGFBP-3 gene expression was detected by Northern blot analysis, but transcripts were less abundant than IGFBP-2 mRNAs. These findings indicate that the expression of multiple BP genes and the secretion of BPs may be a common property of tumour cells.
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PMID:Insulin-like growth-factor-binding protein gene expression and protein production by human tumour cell lines. 137 87

Methods were developed to test angiogenic response to human tumor implants and various biologic agents in the cornea of rabbits and non-human primates (Macaca arctoides). Crude PDGF preparations were found to have significant angiogenic effect. Purified, recombinant PDGF preparations were also effective inhibitors (e.g. pentoxifylline (Px) (which also were found to release PgI2 and t-PA) inhibited human tumor implant induced angiogenesis and reduced spontaneous metastases in 3 transplantable murine tumors (Furth-Columbia Wilms' tumor in Furth-Wistar rats, C-1300 neuroblastoma in A/J mice and HM-Kim mammary carcinoma in Wistar rats) but not in the NIH adenocarcinoma in Balb/c mice. Sodium diethyldithiocarbamate (DDTC), a metal complexing agent with special affinity to copper and anti-thyroid as well as, immune stimulating activity was shown to be anti-angiogenic and to potentiate the effect of Px. The anti-fibrinolytic agents epsilon amino caproic acid (EACA) and tranaxamic acid (t-AMCHA) were anti-angiogenic. DDTC and Px were synergistic from this point of view.
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PMID:Studies on tumor induced angiogenesis. 137 68


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