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Query: UMLS:C0027819 (neuroblastoma)
 27,800 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Neuroblastoma is an embryonal tumour of the sympathetic nervous system with marked heterogeneity in terms of histological maturity and clinical course. A previous study revealed that high tumour levels of the csrc protein, particularly its neuronal isoform (pp60csrcN), correlated with favourable outcome. To test whether this feature reflects a higher degree of neuronal maturation in these tumours, an extended series (47 consecutive neuroblastomas and 10 ganglioneuromas) were analysed for levels of csrc protein isoforms, neuron-specific enolase, and synaptophysin. Immunoblotting and radioimmunoassay techniques were employed. The results were compared with conventional histological signs of neuronal maturation. High pp60csrcN levels were specific for prognostically favourable neuroblastomas and correlated with high neuronal marker levels. However, signs of histological maturation correlated poorly with these parameters. It is therefore concluded that low stage tumours are highly differentiated in biochemical terms despite their frequently immature histology. Furthermore, the clinical usefulness of these biochemical parameters as prognostic markers was compared with established parameters in a multivariate analysis. Stage 4 disease, MYCN amplification, and age above 18 months at diagnosis was the most powerful combination of variables found for predicting a poor outcome. As expected, none of the neuronal differentiation markers investigated could add to the prediction of aggressive disease when compared with this model. However, high expression of pp60csrcN appeared to be useful in predicting long-term survival in high stage infant neuroblastoma.
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PMID:Biochemical evidence for a mature phenotype in morphologically poorly differentiated neuroblastomas with a favourable outcome. 757 41

We have examined the subcellular distribution of synapsins and synaptophysin in density gradients from synapsin- and vector-transfected NG108-15 cells, since we recently found that transfection of synapsin IIb cDNA into neuroblastoma x glioma hybrid cells (NG108-15) resulted in cell lines that had a more neuronal phenotype than controls. The increase in synapsins and synaptophysin in the transfected cells was maximal in the region of the gradient containing small synaptic vesicles. The transferrin receptor, a marker for early endosomes, did not increase in the synapsin-containing fractions in the transfected cells. Secretogranin I, a soluble protein stored in and secreted from large dense cored vesicles, showed a very pronounced increase in the dense regions of gradients from transfected cells. These subcellular fractionation data suggest a possible role for the synapsins in the regulation of synaptic vesicle function.
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PMID:Increase in synaptic vesicle proteins in synapsin-transfected NG108-15 cells: a subcellular fractionation study. 762 29

Twenty cases of olfactory neuroblastoma were available for clinical and histopathological evaluation. The usefulness of immunohistochemistry in the diagnosis of this tumour was investigated and was best achieved using a panel of monoclonal and polyclonal antibodies, notably neuron-specific enolase, PGP 9.5, S-100 protein, synaptophysin and chromogranin A. This study confirmed that immunohistochemistry is a useful adjunct in cases where conventional histology is equivocal.
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PMID:Olfactory neuroblastoma: clinical and pathological aspects. 768 84

The gene defective in Huntington's disease encodes a protein, huntingtin, with unknown function. Antisera generated against three separate regions of huntingtin identified a single high molecular weight protein of approximately 320 kDa on immunoblots of human neuroblastoma extracts. The same protein species was detected in human and rat cortex synaptosomes and in sucrose density gradients of vesicle-enriched fractions, where huntingtin immunoreactivity overlapped with the distribution of vesicle membrane proteins (SV2, transferrin receptor, and synaptophysin). Immunohistochemistry in human and rat brain revealed widespread cytoplasmic labeling of huntingtin within neurons, particularly cell bodies and dendrites, rather than the more selective pattern of axon terminal labeling characteristic of many vesicle-associated proteins. At the ultrastructural level, immunoreactivity in cortical neurons was detected in the matrix of the cytoplasm and around the membranes of the vesicles. The ubiquitous cytoplasmic distribution of huntingtin in neurons and its association with vesicles suggest that huntingtin may have a role in vesicle trafficking.
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PMID:Huntingtin is a cytoplasmic protein associated with vesicles in human and rat brain neurons. 774 55

Esthesioneuroblastoma (ENB; olfactory neuroblastoma) is a rare, locally aggressive neoplasm of the sinonasal area and anterior cranial fossa. The histogenesis of the lesion is not clearly delineated, and the broad histological spectrum of ENB has confounded the issue. The location, histological features (neuropil, Homer Wright, and olfactory rosettes), and reported immunocytochemical reactions (neuron-specific enolase (NSE) and chromogranin (CHR) positivity) suggest that ENB may be a neural or neuroendocrine neoplasm derived from the olfactory membrane. Recent demonstration in two of three metastatic putative ENB cell lines of the 11;22 chromosomal translocation, seen in Ewing's sarcoma (ES) of bone and peripheral neuroectodermal tumors (PNET) of bone and soft tissue, has led to the conclusion that ENB may be closely related histogenetically to PNET. The overwhelming majority of cases of ES and PNET express the protein product of MIC-2, a gene located on the pseudoautosomal region of the X and Y chromosomes. This protein can be identified immunocytochemically by antibodies 12E7, HBA71, and ON13. We studied the expression of MIC-2 using the 12E7 antibody as well as multiple neural markers in 18 ENB samples obtained from the files of The Johns Hopkins Hospital. The patients ranged in age from 19 to 90 years (mean, 47.5; median, 47) and included five men and 13 women. None of the 18 specimens reacted with antibody 12E7, but 16 were positive for NSE, nine reacted to synaptophysin (SYN), and 13 showed antibodies to chromogranin (CHR). Our studies agree with the previous suggestions that ENB is a primitive neural tumor but fail to support the hypothesis that it is a member of the PNET family.
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PMID:Is esthesioneuroblastoma a peripheral neuroectodermal tumor? 777 94

For the sake of discussion, the markedly diversified tumors of the endocrine/neuroendocrine system are classified as those originating in classic epithelial endocrine organs (eg, adrenal cortical adenomas), from the diffuse endocrine cells (eg, jejunal carcinoid tumors), or from clusters of these cells (eg, islet cell tumors); and those arising from neurosecretory neurons (eg, neuroblastoma) or paraganglia (eg, carotid body tumor). Although traditional transmission electron microscopy is useful for identifying neurosecretory or endosecretory granules as such, with few exceptions (eg, insulin-containing granules with a complex paracrystalline core) it is not possible to ascribe a granule type (size, shape, or ultrastructure) to a distinct nosologic entity or secretory product because of their overlapping fine structures in different cell types. Immunoelectron microscopy methods utilizing colloidal gold-labeled secondary antibodies can be used to localize virtually any antigen (peptide or neuroamine) to a specific neurosecretory or endosecretory granule or other cell structure. General endocrine/neuroendocrine cell markers such as neuron-specific enolase, the chromogranins, and synaptophysin are useful in identifying neuroendocrine differentiation in a neoplasm using routine immunohistochemical procedures. The current relevance of the APUD concept of Pearse as well as the biologic importance of endocrine/neuroendocrine secretory products such as bombesin and insulinlike growth factors also are discussed.
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PMID:Tumors of the endocrine/neuroendocrine system: an overview. 791 Jul 11

To determine if immunohistochemistry might aid in the identification of neuroblastomatous foci in composite adrenal tumors, the authors analyzed two examples of composite adrenal pheochromocytoma-neuroblastoma, 18 pure pheochromocytomas, and six pure neuroblastomas using peanut agglutinin and a panel of antibodies directed against neuroendocrine and neural-associated antigens. Pure pheochromocytoma had the following immunopositivity: vimentin 14/18, chromogranin 18/18, synaptophysin 18/18, S100 protein 0/18 (tumor cells), neurofilament 14/18, J1 beta-tubulin (J1) 18/18, microtubule-associated protein-2 13/18, glial fibrillary acidic protein 13/18, and peanut agglutinin 17/18. Pure neuroblastoma reacted positively as follows: vimentin 0/6, chromogranin 5/6, synaptophysin 4/6, S100 protein 0/6 (tumor cells), neurofilament 5/6, J1 6/6, microtubule-associated protein-2 6/6, glial fibrillary acidic protein 1/6, and peanut agglutinin 6/6. Each component of both composite tumors reacted similarly to the pure neoplasms. Although the frequency of positive staining was similar for pheochromocytoma and neuroblastoma, the intensity and pattern differed for several antigens. Pheochromocytoma was diffusely positive for synaptophysin and chromogranin, whereas staining was focal and punctate in neuroblastoma. Microtubule-associated protein-2, J1, and neurofilament antibodies highlighted the fibrillar background of neuroblastoma, which pheochromocytoma lacked. Pheochromocytoma contained focal, ball-like immunoreactivity for glial fibrillary acidic protein and vimentin, which was absent in neuroblastoma. These immunohistochemical distinctions can assist the clinically important recognition of neuroblastomatous foci in composite adrenal pheochromocytoma-neuroblastoma.
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PMID:Immunohistochemical detection of neuroblastomatous foci in composite adrenal pheochromocytoma-neuroblastoma. 804 83

Two patients, 14 and 46 years of age, presented with diffuse, rapidly growing intracerebral tumors leading to death 6 1/2 and 9 1/2 months, respectively, after diagnosis. Histological examination showed sheets of moderate-sized tumor cells with clear cytoplasm and central nuclei interrupted by delicate arciform vasculature, an appearance distinctly different from that of neuroblastoma. Malignant features were present in the form of significant nuclear pleomorphism, numerous mitotic figures, and small foci of necrosis with some suggestion of adjacent pseudo-palisading in one case. Ultrastructural examination showed neuronal differentiation, including prominent neuritic processes, microtubules, dense-core neurosecretory-type granules, and synaptic bouton-like structures containing small, empty-appearing synaptic-type vesicles and synapse-like membrane "thickenings." Immunohistochemistry showed focal immunopositivity for synaptophysin, neurofilaments, neuron-specific enolase, and S100 protein. Immunoreactivity for glial fibrillary acidic protein (GFAP) was found at the margins of the tumors adjacent to some intratumoral blood vessels and in some tumor cells. These tumors seem to occupy a nosological "middle ground" between neuroblastoma and central neurocytoma.
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PMID:Malignant neurocytic tumor. 805 20

We report a case of spinal cord neurocytoma in a 67-year-old man who had experienced a progressive numbness of the left foot during the previous 4 years. Magnetic resonance imaging showed a well-defined intramedullary tumor located at the T10-T11 level. The pathological examination revealed histological characteristics described in neurocytomas. The tumor cells showed a uniform small nucleus and clear or slightly eosinophilic cytoplasm with frequent perinuclear halos, resembling the picture of oligodendroglioma. Some tumor cells exhibited mature ganglion cell appearance. Electron microscopy showed cells with microtubules and dense-core vesicles in their cytoplasm and cytoplasmic process. Immunohistochemically, the majority of tumor cells expressed synaptophysin and neuron-specific enolase. We conclude that this tumor is an exceptional case of neurocytoma located in the spinal cord, and consider that the term neurocytoma can be applied to tumors with neuronal differentiation intermediate between neuroblastoma and ganglioneuroma, even if arising in CNS outside of the intracranial ventricular system.
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PMID:Neurocytoma of spinal cord. 805 8

We report three cases of neurilemoma occurring in the superficial soft tissue of the palm, posterior neck, and flank that closely resembled neuroblastoma by virtue of a predominance of small, round hyperchromatic schwann cells with scant cytoplasm which, in one case formed perivascular rosettes and, in the others, giant rosettes with central collagen cores. Although the diagnosis of neuroblastic tumor was seriously entertained in all cases, the diagnosis of a schwannoma was substantiated by the finding of focal areas of conventional schwannoma and by the typical immunophenotypic profile. All tumors strongly and diffusely expressed S-100 protein but lacked neurofilament protein, protein gene product (PGP), and synaptophysin. Ultrastructurally the cells contained slender cytoplasmic processes invested with basal lamina, but no dense core granules. All were excised conservatively, and none has recurred during the 2, 12, and 27 month follow-up periods.
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PMID:Neuroblastoma-like neurilemoma. 811 94


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