Abstract
To establish the neuroendocrine and neural features of peripheral neuroblastic tumors, a prospectively collected group of 12 neuroblastomas (NB), 2 ganglioneuroblastomas (GNB), and 4 ganglioneuromas (GN) was probed with a panel of monoclonal antibodies (MAbs) to neuroendocrine and neural antigens. All tumors expressed the pan-neuroendocrine markers synaptophysin and chromogranin A. They also showed extensive expression of neuronal antigens, ie, of each of the neurofilament (NF) triplet proteins and of the microtubule-associated proteins (MAPs) MAP2 and tau-protein. However, only in the GNBs and GNs was the pattern of NF phosphoisoforms relatively mature. In the latter tumors glial fibrillary acidic protein (GFAP) and myelin basic protein (MBP) could be demonstrated as well, suggesting the presence of nonmyelinating and myelinating Schwann cells, respectively. The glial markers did not colocalize with the neural markers. On the basis of these data, it was concluded that all peripheral neuroblastic tumors manifest molecular characteristics of neuroendocrine cells and of neurons. The latter were most developed in GNBs and GNs, in which they were accompanied by Schwann cell differentiation in a separate population of cells. The above-outlined neuronal profile of peripheral neuroblastic tumors, including NBs, distinguishes this group of tumors from the much-less neuronally differentiated primitive neuroectodermal tumors of the central nervous system.
Publication types
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Research Support, U.S. Gov't, Non-P.H.S.
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Research Support, U.S. Gov't, P.H.S.
MeSH terms
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Abdominal Neoplasms / metabolism*
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Abdominal Neoplasms / pathology
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Adrenal Gland Neoplasms / metabolism*
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Adrenal Gland Neoplasms / pathology
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Adult
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Antibodies, Monoclonal / analysis
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Biomarkers, Tumor / analysis
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Cell Differentiation
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Cell Transformation, Neoplastic / metabolism
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Cell Transformation, Neoplastic / pathology
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Child
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Child, Preschool
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Chromogranin A
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Chromogranins / immunology
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Chromogranins / metabolism*
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Female
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Ganglioneuroma / metabolism*
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Ganglioneuroma / pathology
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Glial Fibrillary Acidic Protein / metabolism
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Humans
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Immune Sera / immunology
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Immunohistochemistry
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Infant
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Intermediate Filament Proteins / immunology
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Intermediate Filament Proteins / metabolism
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Intermediate Filaments / metabolism
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Male
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Membrane Proteins / immunology
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Membrane Proteins / metabolism*
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Microtubule-Associated Proteins / metabolism
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Myelin Basic Protein / metabolism
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Nerve Tissue Proteins / metabolism*
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Neuroblastoma / metabolism*
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Neuroblastoma / pathology
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Neurons / metabolism*
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Neurons / pathology
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Phosphorylation
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Schwann Cells / cytology
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Schwann Cells / metabolism
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Synaptophysin
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tau Proteins
Substances
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Antibodies, Monoclonal
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Biomarkers, Tumor
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Chromogranin A
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Chromogranins
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Glial Fibrillary Acidic Protein
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Immune Sera
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Intermediate Filament Proteins
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Membrane Proteins
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Microtubule-Associated Proteins
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Myelin Basic Protein
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Nerve Tissue Proteins
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Synaptophysin
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tau Proteins