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)

Twenty-six studies by meta-(131I)-iodobenzylguanidine scintigraphy (131I-MIBG), 26 studies by 67Ga-citrate and 33 99mTc-hydorxymethylene diphosphate (99mTc-HMDP) scintigraphic studies were performed for 10 patients with abdominal neuroblastoma. Comparing the 131I-MIBG images obtained at 24, 48 and 72 h, the 48-h image was the most distinctive for the tumor. Intrabdominal primary lesions, which ranged from bean to fist-size, were visualized in 7/7 cases (100%) by 131I-MIBG, 4/7 cases (57%) by 67Ga-citrate and 4/8 cases (50%) by 99mTc-HMDP before surgery and at diagnosis. In serial follow-up of these patients after starting chemotherapy, 131I-MIGB detected 100% of regressing primary tumors. Studies of 5 postoperative patients showed negative images for the primary tumor in all 3 scintigraphies except one in whom 131I-MIBG was positive, but not 67Ga-citrate or 99mTc-HMDP, for an unresectable residual tumor. 131I-MIBG also detected metastatic lesions not predicted by 67Ga-citrate or 99mTc-HMDP and reflected tumor progression more sensitively than known tumor markers such as urinary vanillylmandelic acid (VMA), homovanillic acid (HVA), serum neuron-specific enolase (NSE) and ferritin. These findings indicate that the 48 hr 131I-MIBG scintigraphy is superior to 67Ga-citrate or 99mTc-HMDP images and to other biochemical markers in monitoring the effect of treatment on neuroblastoma.
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PMID:131I-meta-iodobenzylguanidine scintigraphy in patients with neuroblastoma. 360 63

Statistical procedures were used to estimate lectin receptor distribution on the surface of ascite lymphoma cells, neuroblastoma C-1300 cells and of transformed human T- and B-derived lymphoid cell lines. Relationships between the arithmetic means and mean square variances for sample populations from each cell and ferritin- or colloidal gold-lectin combination were used to define four types of topographical distributions: uniform-ordered, uniform-random, random and clustered.
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PMID:[Evaluation of the distribution of lectin receptors on the surface of tumor and transformed cells using methods of variation statistics]. 360 7

Cellular and humoral markers of malignancy play several roles at many levels in the evaluation and staging of children with cancer. Cytogenetic analysis of constitutional cells can be used to determine the genetic risk of developing certain cancers, such as retinoblastoma and Wilms' tumor in high-risk families. Urinary metabolites of neuroblastoma have been studied not only for accurate diagnostic ability in children with "small round cell" tumors, but as a screen for the presence of the tumor in large normal populations. Markers are valuable as prognostic factors at the time of cancer diagnosis; for example, the use of cell surface antigens and cytogenetics in leukemia phenotyping, leading to alterations in initial therapy. Once found at diagnosis, both specific and nonspecific markers can then be utilized to follow the regression and recurrence of a malignancy, such as serum ferritin in neuroblastoma or lactate dehydrogenase in non-Hodgkin's lymphoma. Presence of cell surface antigens to which monoclonal antibodies can be directed are becoming increasingly helpful in both tumor localization, such as in radioisotope scanning, and in therapeutic intervention, such as in purging autologous bone marrow of malignant cells prior to use as a rescue after massive cytoreduction. Finally, cellular markers have lead to a better understanding of the basic biology of particular neoplasms; for example, gene rearrangements in lymphoma, which will ultimately lead to better diagnostic and therapeutic ability.
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PMID:The use and significance of biologic markers in the evaluation and staging of a child with cancer. 371 38

D-mannose, D-galactose, N-acetyl-D-galactosamine, N-acetyl-D-glucosamine and L-fucose which are sugar determinants of receptors were found on the surface of neuroblastoma cells by means of four carbohydrate-specific lectin groups. Labeling of lectins was performed by horseradish peroxidase, ferritin and colloidal gold. Peculiarities of the lectin receptors distribution on the surface of immature neuroblastoma cells were detected.
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PMID:[Localization of lectin receptors on the surface of C1300 neuroblastoma cells]. 375 84

Recently accumulated data on the biology of the tumors of neuroblastoma group are reviewed. Histopathological classification system developed by Shimada et al, elevated levels of serum neuron-specific enolase and ferritin, and gene abnormalities including partial deletion of the short arm of chromosome #1 and N-myc gene amplification are discussed as well as their prognostic significance. Also, the current activities of CCSG (Childrens Cancer Study Group) neuroblastoma studies are briefly reported.
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PMID:[Neuroblastoma; biology and prognostic factors]. 380 Apr 8

That ferritin, an iron storage protein, can be produced by neuroblastoma cells raises the possibility that iron may have some role in promoting tumor cell growth. To explore this possibility, we studied the effects of desferoxamine, a compound which chelates iron, on viability of CHP 126 and CHP 100, two human neuroblastoma cell lines. Cells (5 X 10(4)) were incubated with graded amounts of desferoxamine or ferrioxamine, an iron-saturated analogue of desferoxamine. Within 5 days of exposure to 60 microM desferoxamine, approximately 90% of cells from each of these cell lines were dead. This effect was dose dependent, was not seen with ferrioxamine, and could be prevented by coincubation with greater than stoichiometric amounts of ferric citrate. As determined by binding of OK-T9, desferoxamine also resulted in increased expression of receptors for transferrin, an iron transport protein. Desferoxamine had only minimal effects on viability of several non-neuroblastoma cell lines. These results suggest that iron is required for growth of neuroblastoma and that desferoxamine has potent, specific, antineuroblastoma activity in vitro.
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PMID:Antineuroblastoma activity of desferoxamine in human cell lines. 381 70

These studies suggest that a) the levels of serum ferritin are closely related to the prognosis of patients with neuroblastoma; b) the increased amounts of ferritin in the serum of patients with neuroblastoma are, in part, derived from the tumor; c) isoferritins from neuroblastoma cells exert adverse effects on the host immune response and host defenses; and d) therefore, isoferritins released from tumors may, in part, be responsible for the poor prognosis of patients with elevated levels of serum ferritin.
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PMID:Serum ferritin as a prognostic indicator in neuroblastoma: biological effects of isoferritins. 387 75

Ferritin was measured in sera obtained at diagnosis from 241 patients with neuroblastoma to determine (a) the incidence of elevated ferritin and (b) the relationship between ferritin level and outcome. Ferritin was infrequently elevated in sera from patients with Stages I and II disease but was abnormally elevated in 37 and 54% of those with Stages III and IV neuroblastoma, respectively. The mean and median levels for each stage were compared and were highest for Stages III and IV disease. Analysis of progression-free survival for children with Stages III and IV disease indicated that elevated ferritin was associated with a significantly poorer prognosis than was normal ferritin and that this correlation was independent of stage and age at diagnosis. Progression-free survival at 24 months of follow-up for patients with Stage III disease with normal ferritin was 76% and with elevated ferritin was 23%. For those with Stage IV disease, progression-free survival was 27 and 3% with normal and elevated ferritin, respectively. We conclude that determination of the level of ferritin in serum at diagnosis is useful for selecting appropriate therapy for patients with Stage III neuroblastoma. Those with normal ferritin (63% of patients) have a good outcome with current therapy, but those with elevated ferritin (37%) do poorly and require more effective therapy. Although ferritin defines subgroups with Stage IV disease, the outcome of all groups must be improved.
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PMID:Prognostic importance of serum ferritin in patients with Stages III and IV neuroblastoma: the Childrens Cancer Study Group experience. 398 11

Serial serum ferritin (SF) levels were measured in 36 patients with neuroblastoma seen at Memorial Sloan-Kettering Cancer Center (MSKCC) between January 1981 and December 1982. The significance of the associations among SF, stage and extent of disease, number of blood transfusions, liver function, serum iron (Fe), total iron-binding capacity (TIBC), and transferrin saturation was investigated. Although a dominant statistical correlation was found between SF and number of blood transfusions, the results suggest that amount of disease contributes to increasing SF levels. Serum ferritin levels increased on average in a linear fashion with number of blood transfusions in patients free of disease or with minimal disease. In patients with bulky disease, this increase was exponential (p value less than 0.01). Application of a reverse hemolytic plaque assay to the analysis of ferritin secretion by cells demonstrates that tumor cells do secrete ferritin in vitro.
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PMID:Ferritin in neuroblastoma. Impact of tumor load and blood transfusions. 402 55

Elevated serum ferritin levels without a corresponding increase in tissue iron storage have been observed in patients with certain cancers. Increased synthesis of ferritin by cancer cells has also been reported. In order to see whether similar phenomena occurred in patients with neuroblastoma, we have screened serum ferritin levels in 58 children with neuroblastoma by counterelectrophoresis using antibody to human ferritin. Increased ferritin levels in serum, positive by counterelectrophoresis (greater than or equal to 400 ng/ml), correlated well with the presence of active disease (p less than 0.001 by Fisher's exact 2 X 2 test). A longitudinal study of serum ferritin levels in 34 of the 58 patients showed the same association of elevated serum ferritin with active disease; a return of ferritin levels to the normal ranges coincided with remission. Primary neuroblastoma tumors and cells from neuroblastoma cell lines contained ferritins with the electrophoretic characteristics different from normal liver ferritin. Supernatant fluids from six neuroblastoma cell lines grown in culture also contained ferritin. These findings suggest that the increased ferritin in the serum of patients is derived from the tumor. The serum ferritin level could be used as indicator of disease activity and as a guide to therapy.
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PMID:Serum ferritin as a guide to therapy in neuroblastoma. 624 92


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