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)

Neuroblastoma and peripheral PNETs both are typical examples of wide group of small round blue cell tumors of childhood. Matured neuroblastoma show typical clinical presentation and easy to interpret microscopic picture. Unfortunately in everyday practice much commonly appeared less differentiated neuroblastomas with difficult to predict clinical behavior and impossible to diagnose in routine stain histologic view. Peripheral PNETs are found as morphologically similar entities to some neuroblastoma subtypes but they are treated as separate CD99 positive group of tumors with different biology and clinical behavior. The aim of our study was to estimate the usefulness of neural markers expression (Neuroblastoma Marker, neurospecyfic enolase and neurofilaments) in routine separation between neuroblastoma tumors and pPNETs and between neuroblastoma subtypes according to currently used classification of those entities. We investigated 63 tumor tissue samples and found differences in expression of investigated markers between both neuroblastoma subgroups and neuroblastoma group of tumors and pPNETs.
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PMID:Estimation of diagnostic value of chosen markers of neural differentiation in neuroblastoma group of tumors and pPNETs. 1939 85

Human umbilical cord blood (HUCB) provides a source of progenitors for cell therapy. We isolated and characterized an HUCB-derived population of progenitors (HUCBNP), differentiated toward neuronal phenotype by human neuroblastoma-conditioning medium (CM) and nerve growth factor (NGF), which have been found to confer neuroprotection toward hypoxia-mediated neuronal injury. This study investigated whether interferon-gamma (IFN-gamma) contributes to HUCBNP differentiation. IFN-gamma was detected in the CM used for the induction of differentiation of HUCBNP and a neutralizing antibody of IFN-gamma significantly inhibited either IFN-gamma or CM-induced differentiation. Transcriptome analysis of CM-differentiated HUCBNP, identified 86 genes as highly upregulated, among them 25 were IFN-induced (such as 2',5'-oligoadenylate synthetase 1 and 2, IFN-induced protein and transmembrane proteins, STAT1 (IFN-gamma-receptor signal transducer and activator of transcription) and chemokine C-X-C motif ligand 5). Treatment of HUCBNP with human recombinant IFN-gamma, inhibited cell proliferation in a dose-dependent manner. IFN-gamma (1-100 ng/ml) enhanced neuronal differentiation, expressed by neurite outgrowths and increased expression of the neuronal markers beta-tubulin III, microtubule-associated protein 2, neuronal nuclei, neurofilament M and neuronal-specific enolase. IFN-gamma additively cooperated with NGF to induce the differentiation of HUCBNP. These data indicate that IFN-gamma promotes neuronal differentiation of HUCB-derived progenitors, proposing its use in future protocols towards cell therapy.
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PMID:Interferon-gamma-induced neuronal differentiation of human umbilical cord blood-derived progenitors. 1945 27

A 35-year-old lady presented with recurrent episodes of painful right upper lid swelling and ptosis of 6 months' duration. The symptoms would subside with oral antibiotics and painkillers. She had also been undergoing treatment for one year for multiple painful osteolytic lesions in the long bones and axial skeleton for which no definite diagnosis had been made, despite several radiological and serological investigations having been performed. Computed tomographic scan showed a soft tissue lesion in the superolateral part of the right orbit with erosion of the adjacent bone. Lacrimal gland biopsy showed non-specific inflammatory changes. She was referred for full work-up to exclude lymphoma and metastasis. Magnetic resonance imaging revealed an enhancing soft tissue mass in the right anterior cranial fossa eroding the lateral orbital wall and extending into the orbit. Another mass encased the superior sagittal sinus. Bone marrow biopsy showed near total replacement of marrow cells by a round cell tumour. Immunohistochemical studies revealed that the tumour cells expressed neuron specific enolase and chromogranin. The pathological findings suggested a diagnosis of neuroendocrine tumour though neuroblastoma could not be unequivocally ruled out. (131) I-metaiodobenzylguanidine scan showed no uptake. Radiologic studies suggested multiple bony metastases. No other site of primary tumour was found so there was a strong possibility of it being a primary orbito-cranial neurogenic tumour. Neuroblastomas and neuroendocrine tumours are very rare in adults. The orbit is an unusual site for a primary of this nature. In addition, it is quite difficult to make a specific diagnosis in a less differentiated tumour.
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PMID:Rare orbitocranial tumour in an adult. 1983 15

A 35-year-old lady presented with recurrent episodes of painful right upper lid swelling and ptosis of 6 months' duration. The symptoms would subside with oral antibiotics and painkillers. She had also been undergoing treatment for 1 year for multiple painful osteolytic lesions in the long bones and axial skeleton for which no definite diagnosis had been made, despite several radiological and serological investigations having been performed. Computed tomographic scan showed a soft tissue lesion in the superolateral part of the right orbit with erosion of the adjacent bone. Lacrimal gland biopsy showed non-specific inflammatory changes. She was referred for full work-up to exclude lymphoma and metastasis. Magnetic resonance imaging revealed an enhancing soft tissue mass in the right anterior cranial fossa eroding the lateral orbital wall and extending into the orbit. Another mass encased the superior sagittal sinus. Bone marrow biopsy showed near total replacement of marrow cells by a round cell tumour. Immunohistochemical studies revealed that the tumour cells expressed neuron specific enolase and chromogranin. The pathological findings suggested a diagnosis of neuroendocrine tumour though neuroblastoma could not be unequivocally ruled out. A(131) I-metaiodobenzylguanidine scan showed no uptake. Radiologic studies suggested multiple bony metastases. No other site of primary tumour was found so there was a strong possibility of it being a primary orbito-cranial neurogenic tumour. Neuroblastomas and neuroendocrine tumours are very rare in adults. The orbit is an unusual site for a primary of this nature. In addition, it is quite difficult to make a specific diagnosis in a less differentiated tumour.
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PMID:Rare orbitocranial tumour in an adult. 2656 7

Enteric glial cells (EGCs) are essential in the control of gastrointestinal functions. Although lesions of EGCs are associated with neuronal degeneration in animal models, their direct neuroprotective role remains unknown. Therefore, the aims of this study were to demonstrate the direct neuroprotective effects of EGCs and to identify putative glial mediators involved. First, viral targeted ablation of EGCs in primary cultures of enteric nervous system increased neuronal death both under basal conditions and in the presence of oxidative stress (dopamine, hydrogen peroxide). Second, direct or indirect coculture experiments of EGC lines with primary cultures of enteric nervous system or neuroblastoma cell lines (SH-SY5Y) prevented neurotoxic effects induced by oxidative stress (increased membrane permeability, release of neuronal specific enolase, caspase-3 immunoreactivity, changes in [Ca(2+)](i) response). Finally, combining pharmacological inhibition and mRNA silencing methods, we demonstrated that neuroprotective effects of EGCs were mediated in part by reduced glutathione but not by oxidized glutathione or by S-nitrosoglutathione. Our study identified the neuroprotective effects of EGCs via their release of reduced glutathione, extending their critical role in physiological contexts and in enteric neuropathies.-Abdo, H., Derkinderen, P., Gomes, P., Chevalier, J., Aubert, P., Masson, D., Galmiche, J.-P., Vanden Berghe, P., Neunlist, M., Lardeux, B. Enteric glial cells protect neurons from oxidative stress in part via reduced glutathione.
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PMID:Enteric glial cells protect neurons from oxidative stress in part via reduced glutathione. 1990 78

We saw in consultation a biopsy specimen from a 6-year old girl with anaplastic lymphoma kinase (ALK)-positive anaplastic large cell lymphoma (ALCL). The tumor arose in soft tissue of the neck, and diagnostic tissue was obtained by core needle biopsy. Histologically, the neoplasm was cellular without pattern. Immunohistochemical workup with a large panel of antibodies at another institution showed immunoreactivity for NB84 and neuron specific enolase (dim). Antibodies specific for CD3, CD20, and CD45/LCA were negative; CD30 or ALK were not assessed. Electron microscopy showed cytoplasmic structures thought to be neurosecretory granules. The neoplasm was interpreted initially as a neuroblastoma. At the time of our review, we considered the possibility of ALCL. Immunohistochemical analysis for CD30 showed bright, uniform expression and ALK was positive in a nuclear and cytoplasmic pattern, confirming the diagnosis of ALK+ ALCL. The purpose of this review is to discuss ALK+ ALCL and many of the other entities included under the rubric of small round blue cell tumor, with a focus on tumors that occur in children.
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PMID:Anaplastic large cell lymphoma: another entity in the differential diagnosis of small round blue cell tumors. 1991 80

An analysis of enolase expression during differentiation of neuroblastoma clones in homogeneous culture is presented. The enolases expressed in these neuroblast-like cells are identical to those of mouse brain with respect to the examined properties. Our biochemical investigation has premitted us to demonstrate formally that neuroblastoma cells undergo a transition from the embryonic ?? form to the neuronal ?? form and contain both enolases as well as the ?? hybrid form during maturation. These results suggest that the same phenomenon must exist in vivo for neuroblasts. In neuroblastoma cells, an increase in both ?? and ?? neuron specific enolases is related to cell maturation and expression of the ?? form precedes that of the ?? form during differentiation. Modulation of neuronal enolase activities is similar in the various conditions of differentiation studied and appears not to be necessarily related with morphological differentiation, although concomitant with an arrest of cell division. The evolution of specific neuronal enolases in neuroblastoma cells parallels that observed in vivo, in brain from embryonic day 15 to post-natal day 7. Moreover, at least one treatment (dimethylsulfoxide) causes an important decrease in the high specific ?? activity of these cells as occurs in vivo. This enolase can therefore also be considered as a biochemical marker for neuroblastoma maturation. As observed with other markers and other cell types, neuroblastoma cells in culture express an immature biochemical differentiation of the enolase isozymes.
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PMID:Transition between isozymic forms of enolase during in vitro differentiation of neuroblastoma cells-II. 2048 27

A clonal ascited type cell, NAs-1, was obtained in culture from a mouse neuroblastoma C1300. The cells were adapted to anchorage-independently grow in the flask by the in vitro-in vivo alternate passage technique, and retained the ability of growing and producing ascites fluid when intraperitoneally injected into mice. Although the majority of growing cells in culture medium showed a small and round cell shape without any neuronal process, occasionally non-specific attachment onto the flask surface was observed, but devoid of the extrusion of processes. Karyotype analysis showed a homogeneous chromosome number, 40, with a marker chromosome [t(13:16)] and a minichromosome. Catecholamines, norepinephrine and dopamine, were found in the cell extracts and the contents of dopamine was particularly high as shown in another catecholaminergic neuroblastoma cell, N1E-115. Neuron specific enolase (?-subunit) was also detected. The treatment of the cells by dibutyryl cyclic AMP, prostaglandin E(1), or BL191 (phosphodiesterase inhibitor) induced the biochemical differentiation in terms of catecholamine and cyclic AMP contents, but failed to promote typical morphological differentiations including the extension of process or the significant promotion of adherence onto the flask surface.
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PMID:In vitro and in vivo growth characteristics of a new ascites-type neuroblastoma cell from mouse C1300 neuroblastoma. 2049 95

A specific event which accompanies the terminal differentiation of most neurons is the isozymic enolase transition from the ?? form to the ?? and ?? adult neuronal forms. It is partly expressed during maturation of a mouse neuroblastoma clonal cell line (NIE-115). We demonstrate, in these cell cultures, that the significant increase in the concentration of ? gene product, expressed as ?? enolase during differentiation, is due to a parallel and similar increase in its synthesis. The capacities of poly (A)(+) RNA from undifferentiated and differentiated cultures to direct the synthesis of ? gene product were compared in a reticulocyte cell-free protein-synthesizing system. This translating capacity is stimulated in the same proportion as the rate of synthesis of ? antigen in differentiating cell cultures. Therefore the increase in the level of ? protein (expressed mostly as ?? enolase) during differentiation results from the increased translating activity or relative amount of ?mRNA.
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PMID:Regulation of neuron-specific enolase isozyme levels during differentiation of murine neuroblastoma cell cultures. 2049 98

Neuroblastoma (NB) is a common malignancy in children, but rarely occurs in adults. Accepted unfavorable prognostic factors include age over one year, low histological grade and advanced stage, MYCN amplification, chromosomal aberrations, elevations of neuron specific enolase and lactate dehydrogenase, and increased catecholamine metabolites in urine or serum. In adults, abdomen/retroperitoneum are the primary sites and in children the adrenal gland. We report a 38-year old civil servant who presented at our urology clinic on the 21(st) of December 2007 with a six month history of right flank dull pain which was worse on walking and relieved by rest, hypertension and a large right retroperitoneal mass. Tumor resection revealed a grade III NB. Chemotherapy using a combination of vincristine, adriamycin and cyclophosphamide was started. Follow-up showed regression of the mass initially with a relapse after patient absconded for three months. He resurfaced with new masses and he had a repeat chemotherapy with disappearance of the masses and is currently undergoing further treatment. To our knowledge this is the only report of NB in an adult registered so far in Nigeria and perhaps the whole of Africa. Currently, there are no standard treatment guidelines for patients with NB in adulthood. This study emphasizes the need for a standard treatment regime for adult onset neuroblastoma and its recognition as a possible differential in intra-abdominal mass in adults.
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PMID:Neuroblastoma occurring in a 38-year old Nigerian man: a rare finding. 2113 86


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