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Neuroblastoma Authors: Doctors Matthias Schell 1 and Christophe Bergeron Creation date: October 2003 Scientific Editor: Professor Thierry Philip 1 Centre Léon Bérard, Service de Pédiatrie, 69373 Lyon cedex 08, France. schell@lyon.fnclcc.fr Abstract Key-words Disease name and synonyms Definition Differential diagnosis Etiology Clinical description Outcome Diagnostic methods Epidemiology Treatment Unresolved questions References Abstract Neuroblastoma is a malignant tumour of neural crest cells which give rise to the sympathetic nervous system. It is a childhood tumour occurring in infants and young children (5 years and less in 90% of all cases) that accounts for 8 to 10% of pediatric cancers. It may arise at any site in the sympathetic nervous system, most commonly ...

Original Article Secondary Myelodysplastic Syndrome and Leukemia Following 131 I-Metaiodobenzylguanidine Therapy for Relapsed Neuroblastoma Brian Weiss, MD, Amish Vora, MD, John Huberty, MSc, Randall A. Hawkins, MD, PhD, and Katherine K. Matthay, MD Purpose: To describe three patients with secondary leukemia after treatment with 131 I-metaiodobenzylguanidine (MIBG) for neuro- blastoma. Methods: Of 95 children with refractory neuroblastoma treated with 131 I-MIBG at UCSF, 3 have been identified with secondary myelodysplasia/leukemia. The case records and bone marrow re- sults were reviewed, along with a review of the literature. Results: Three patients developed secondary myelodyspla- sia/leukemia, at 7, 11, and 12 months following 131 I-MIBG therapy. Cytogenetic abnormalities included -7q/-5, -7/+2q37, ...

Detection of Amplification in Neuroblastoma Using Polymerase Chain Reaction and Its Impact on Survival A P RATAP , S S INHA , DK G UPTA Molecular biology has revolutionized the medicine and added a new dimension to our knowledge of disease processes, especially in the field of oncology. Proto oncogenes are proteins normally present in the genome and they may be converted to oncogenes by mutations, rearrangements, translocation and deletion. One such protooncogene is the N-myc proto-oncogene seen in neuroblastoma . Amplification of the N-myc oncogene identifies a group of patients who have a poor prognosis. At present, it is generally agreed that the amplification of N-myc is still the most important prognostic factor to be investigated in every patient with neuroblastoma because of it’s reliability as a prognostic indicator. ...

Neuroblastoma Incidence in Illinois: 1986-1994 Neuroblastomas are childhood tumors of the autonomic nervous system. The annual incidence rate among children under 15 years of age in the United States is nine to 11 cases per million. In Illinois, according to data reported to the Illinois State Cancer Registry for 1986 through 1994, the annual incidence rate was 10.3 per million. Table 1 lists numbers of neuroblastoma incidence among children less than 15 years of age by county in Illinois from 1986 though 1994. As one would expect, counties with a large population, such as Cook and Du Page, tend to have more cases. In order to detect unusually high incidence of neuroblastoma , numbers in Table 1 need to be related to their population denominators, compared with the expected number of cases based on the overall neuroblastoma incidence in Illinois, and expressed in terms of probabilities. This was done by calculating ...

Abstract. The aim of this study was to investigate gene expression changes in disseminated neuroblastoma by cDNA array technique. Three stage IV neuroblastomas and one neuroblastoma cell line (SK-N-FI) were analyzed. Expression profiles were confirmed by semiquantitative RT-PCR and in some instances by Northern blotting. Comparison of expression profiles identified several genes which were highly expressed as well as some which were down-regulated in tumor samples relatively to SK-N-FI cells. The tumors studied lacked N- myc overexpression, while showing up-regulation of basic trans-cription factors, growth factors and receptors such as NSEP, c-myc binding protein MM1, thymosin ß10 (TMSB10), TNF-R superfamily member 10A (TNFRSF10A) and FZ9. These results suggest that cDNA array technology is a powerful tool to identify a set of genes involved in neuroblastoma genesis and progression. Thus, the ...

R ulation of Growth and Difterentiated Function in T ukaryote Cells. edited by G. P. Talwar. Raven Press, New York, 0 1975. Coding of Neural Information by Neuroblastoma Cells Marshall W. Nirenberg Laboratory of Biochemical Genetics, National Heart a& Lung Institute, National lnsfitutes of Health, Bethesda, Maryland 20014 Numerous neuroblastoma clones originating from mouse tumor C 1300 were characterized with respect to neuronal properties such as neuro- transmitter metabolism, cell receptors and response to transmitters, and action potential reactions. Three types of clones were found with respect to enzymes for transmitter synthesis: clones with choline acetyltransferase activity, which synthesize acetylcholine; clones with tyrosine hydroxylase activity, which synthesize catechols; and clones without these enzyme activities. ...

Neuronal Intermediate Filament Protein -Internexin Facilitates Axonal Neurite Elongation in Neuroblastoma Cells Thomas B. Shea* and Mary Lou Beermann Department of Biological Sciences, Center for Cellular Neurobiology and Neurodegeneration Research, University of Massachusetts at Lowell, Lowell We examined the localization and role of -IN vs. other neuronal intermediate filaments before and during differentiation. Vimentin but not -IN localized within filopodia-like neurites of undifferentiated cells. During differentiation, -IN immunoreactivity accumulated within axonal neurites following vimentin but, as previously describe in neurons in situ, before the appearance of NF-L. We therefore manipulated -IN synthesis, accumulation, and function in attempts to determine whether or not this intermediate filament species played a role in axonal development. Intracellular delivery of anti- -IN antisense ...

24 p53 response and function in neuroblastoma cell lines. B. Nelson, M. Elliott, H.P. McDowell, M.R.White University of Liverpool, SOBS, Life Sciences Building, Crown Street, Liverpool, L69 7ZB. INTRODUCTION Neuroblastoma is the most common solid childhood cancer and the prognosis for this condition remains poor. p53 mutations are rare in neuroblastoma , suggesting possible defects downstream of p53 in the apoptotic pathway. METHODS Using neuroblastoma cell lines we have quantified p53 expression and its downstream targets by Western Blotting, after treatment with DNA damaging agents. The transcriptional activity of p53 was determined using a luciferase reporter gene. Real time fluorescence confocal microscopy of living cells and flow cytometry were used to characterise the timing and nature of cell death in response to DNA damaging agents. RESULTS 1. The effect ...

Proc. Nat. Acad. Sci. USA Vol. 69, No. 1, pp. 256263, January 1972 Neurotransmitter Synthesis by Neuroblastoma Clones (neuroblast difTerentiation/cell culture/choline acetyltransferase/acetylcholinesterase/ tyrosine hydroxylase/axons-dendrites) TAKEHIKO XhIANO, ELLIOTT RICHELSON, AND hIhRSH;ILL NIRESISERG L&orator), of Biochemical Genetics, Nrrtionnl Hemt and Lung Institute, Ndotd Institutes of Henlth, Bethesdn, JIxylantl 20014 ABSTRACT Neuroblastoma clones were examined for choline acetyltransferase (EC 2.3.1.6), tyrosine hydroxyl- ase (EC 1.14.3.a), acetylcholinesterase (EC 3.1.1.?), and also for neurite formation. One clone does not form axons or ...

Proc. Nat. Amd. Sci. USA Vol. 71, No. 9, pp. 3474-3477, September 1974 r-- A Neuroblastoma x Glioma Hybrid Cell Line with Morphine Receptors ! (cell membranes/narcotics/neurobiology/tissue culture) WERNER A. KLEE AND MARSHALL NIRENBERG Laboratory of General and Comparative Biochemistry, National Institute of Mental Health and the Laboratory of Biochemical Genetics, National Heart and Lung Institute, National Institutes of Health, Beth&a, Maryland 20014 Contributed by Marsh&l Nirenberg, June l&197.4 ABSTRACT A neuroblastoma X glioma hybrid ceR line with welldeveloped neural properties was found that has high-affinity morphine receptors. The average cell contains approximately 3 X 106 receptors. In contrast, parent ...