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Results for “Neuroectodermal Tumors, Primitive, Peripheral”

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At least 595 records · Page 33Linked to original sources
PubMed1981

Esthesioneuroblastoma.

A retrospective review of the clinical records of 31 patients with esthesioneuroblastoma is presented. A bimodal age distribution was noted. The tumor is extremely rare among blacks. Most patients presented with locally advanced disease. However, regional and distant metastases at the time of initial diagnosis are uncommon. Local recurrence at the primary site was very common, and this reflects either the conservative initial surgical treatment employed or the multicentric nature of the tumor. Cervical lymph node metastasis is present in less than 10 percent of patients at the time of diagnosis. Survival rates were better in patients with early stages of disease. From this study we conclude that the current management of these tumors should consist of a combination of radiation and surgery. Radical resection followed by postoperative radiotherapy appears to be the treatment combination of choice. The 5 year survival rate in our series was 52 percent.

Adolescent↗
PubMed1976

Nervous system antigen-5, an antigenic cell surface component of neuroectodermal origin.

The antigenic cell surface component NS-5 (nervous system antigen-5) is recognized by antiserum raised in C3H.SW/Sn mice against cerebellum of 4-day-old C57BL/6J mice. When analyzed in the cytotoxicity test the antiserum detects a cell surface antigen or set of antigens present not only an cerebellum but also other parts of the central nervous system, including retina, as well as on mature spermatozoa and to a lesser degree on kidney. All other non-neural tissues tested, liver, splee, thymocytes, muscle, testis, adrenal gland and epidermis do not express detectable amounts of the antigen. Among seven murine tumors of the nervous system, medulloepithelioma shows high levels of NS-5 expression, whereas neuroblastoma Cl300, glioma G26, glioblastome, ependymoblastoma, ependymoblastoma EPA and glioblastoma G26l do not carry detectable NS-5. All mouse strains tested (C57BL/6J, C3H.SW/Sn, C3H/HeDiSn, A/J, AKR/J, BALB/cJ and DBA/2) express similar levels of NS-5. The antigen is demonstrable not only on postnatal day 4 neural tissue, but also in lower amounts on adult nervous system. On embryonic day 9, the earliest stage tested, and at all subsequent stages during embryonic development, NS-K is already present in brain and spinal cord, but not in gut.

Age Factors↗
PubMed1990

The cholecystokinin gene is abundantly co-expressed with gastrin-releasing peptide, enkephalin and neuropeptide Y genes in a clonal human neuroepithelioma cell line.

The cholinergic human neuroepithelioma cell line SK-N-MCIXC expressed mRNAs for the neuropeptides cholecystokinin (CCK), neuropeptide Y, gastrin-releasing peptide (GRP) and enkephalin. The CCK transcript of about 800 nt was present at very high levels and CCK-like peptides immunoreactive to a C-terminal CCK octapeptide antiserum were present in the cell line and its medium. This clonal neuronal cell line provides a unique model system to identify cis- and trans-acting factors responsible for neuron-specific expression and regulation of the CCK gene. Furthermore, the pluripotent properties of the undifferentiated cell line may open studies on neuronal differentiation at the level of co-expression of neuropeptides and transmitters.

Blotting, Northern↗
PubMed1993

Regulation of CCK mRNA in the human neuroepithelioma cell line SK-N-MCIXC in response to second messenger activators.

Regulation of cholecystokinin (CCK) expression was studied in the human neuroepithelioma cell line SK-N-MCIXC. The cells were treated with the phosphodiesterase inhibitor isobutyl-methylxanthine and the tumor promoting phorbol ester, phorbol-12-myristate 13-acetate; activators of the cyclic AMP (cAMP) and protein kinase C (PKC) second messenger pathways, respectively. Levels of CCK mRNA were determined after 6, 12 and 24 hour drug treatments, with Northern blot analysis using human CCK cDNA hybridization probes. Activation of both cAMP and PKC second messenger pathways increased CCK mRNA levels in SK-N-MCIXC cells. These results indicate that the levels of CCK mRNA in SK-N-MCIXC cells are regulated by cAMP and PKC dependent mechanisms.

1-Methyl-3-isobutylxanthine↗
PubMed1991

Effects of target antigen density on the efficacy of immunomagnetic cell separation.

Immunomagnetic cell separation uses binding of an antibody to its epitope to identify the target cell, which is then removed by attachment to an anti-immunoglobulin-coated paramagnetic bead, and passage through a magnetic field. This method has previously been shown to be less sensitive to the effects of low target antigen density than are other cell elimination methods, such as complement-mediated lysis. In this paper we demonstrate that, with certain antibody/target cell combinations, the efficiency of immunomagnetic depletion can be adversely affected by high expression of the target antigen. This can occur by two non-mutually exclusive mechanisms. These are (i) steric hindrance of bead binding due to crowding of monoclonal antibodies on the cell surface; and (ii) binding of the monoclonal antibody molecule in a configuration that is poorly-accessible to the anti-immunoglobulin immobilized on the microspheres. The predominant effect operating in any system can be determined by analysis of the cells remaining after the separation procedure. In both cases pre-attachment of the monoclonal to the beads results in improved separation efficiency. These results emphasize the necessity of optimizing experimental conditions in each system that is investigated.

Antibodies, Monoclonal↗
PubMed1992

Insulin desensitized beta 1-adrenergic receptor-mediated stimulation of adenylyl cyclase in SK-N-MC cells.

Receptor cross-talk is an emerging field which investigates cross-regulation between distinct classes of receptors. In the present work, we investigated the influence of activating the insulin receptor, a tyrosine kinase receptor, on beta-agonist activation of adenylyl cyclase, which is mediated by a G protein-linked receptor. Treatment of SK-N-MC neuroepithelioma cells with insulin generated a marked attenuation of beta 1-adrenergic receptor-mediated stimulation of adenylyl cyclase. This effect required nanomolar concentrations of insulin, occurred within minutes of exposure of these cells to insulin, and did not result from down-regulation of beta-adrenergic receptors. Insulin alone reduced the maximal isoproterenol-mediated stimulation of adenylyl cyclase by 50%, while the co-addition of the phosphatase inhibitor sodium vanadate increased the magnitude of insulin inhibition to 90%. Insulin provides an additional avenue for heterologous desensitization of beta-adrenergic receptors and their transmembranal signalling pathway.

Adenylyl Cyclases↗
PubMedDate not supplied

Ophthalmic striated muscle neoplasms.

Rhabdomyosarcoma, the most common primary malignant childhood orbital tumor, is composed of neoplastic striated muscle cells (rhabdomyoblasts) in various stages of differentiation and in patterns suggestive of neoplastic analogs of normal muscle embryogenesis. Orbital rhabdomyosarcoma is most commonly seen in children and adolescents, the average age of onset of symptoms being 7.8 years. The tumor usually presents as a rapidly evolving exophthalmos, often associated with drooping of the upper eyelid. A mass is palapable in only 25% of cases, loss of central vision at the time of presentation is uncommon, and laboratory studies are often of little help in diagnosis. The best diagnostic aid is a high index of suspicion whenever one sees a rapidly progressive exophthalamos in a child. Orbital rhabdomyosarcoma is almost always of the embryonal type, believed to originate in the orbital soft tissues from undifferentiated pluripotential embryonic mesenchyme. In the past, orbital exenteration has been the primary therapy. Review of 162 literature cases of orbital rhabdomyosarcoma, generally treated by unassisted surgery, revealed that only 25% of the patients survived 3 or more years. Recently, it has been shown that radiation therapy, alone or combined with chemotherapy, can be successful. A multidisciplinary approach, utilizing surgery, radiation therapy and chemotherapy has also been advocated. Both approaches appear to offer greater survival than unassisted orbital exenteration. The possibility of primary radiation therapy is extremely promising; if it becomes increasingly effective, a mutilating surgical procedure may become obsolete.

Adolescent↗
PubMedDate not supplied

Monoclonal antibodies to intermediate filament proteins: diagnostic specificity in orbital pathology.

Intermediate filaments derived from different cell types are antigenically distinct. Monoclonal antibodies to human intermediate filament proteins can, therefore, be used as tissue-specific reagents capable of distinguishing cell type in poorly differentiated neoplasms. We report a case demonstrating the specificity of antiintermediate filament protein antibodies in establishing a difficult orbital diagnosis of esthesioneuroblastoma.

Antibodies, Monoclonal↗
PubMed1995

Dysembryoplastic neuroepithelial tumor: report of three cases.

BACKGROUND: Dysembryoplastic neuroepithelial tumor (DNT) is one of the mixed neuronal-glial tumors that should be differentiated from low-grade gliomas. The recognition and correct diagnosis of DNT is important because this tumor is curable by excision. METHODS: We report three cases of histologically proven DNTs with various image studies performed preoperatively and describe characteristic findings of them, as well as those of previous reports. RESULTS: In our three cases, computed tomography (CT) showed a well-defined, nonenhancing hypodense area. Magnetic resonance imaging (MRI) demonstrated a cortical mass of hypointensity on T1- and of marked hyperintensity on T2-weighted images. I-123-IMP or Tc-99m-HMPAO single-photon emission computed tomography (SPECT) images showed marked hypoperfusion of the lesion. Thallium-SPECT showed no uptake. CONCLUSIONS: I-123-IMP, Tc-99m-HMPAO, and Thallium-SPECT images, as well as CT and MRI, may be helpful in distinguishing DNT from low-grade gliomas.

Adult↗
PubMed1993

Processing, release and metabolism of cholecystokinin in SK-N-MCIXC cells.

The human cholinergic neuroepithelioma cell line SK-N-MCIXC, which expresses high levels of cholecystokinin (CCK) mRNA and secretes intact CCK into the media, was used to examine CCK processing and metabolism. Our data provide evidence for the existence of specific candidate processing enzymes in SK-N-MCIXC cells which may be involved in processing proCCK in the brain and indicate that SK-N-MCIXC cells provide a model system for studying the regulation of these enzymes. mRNAs for the intracellular processing enzymes, prohormone convertase 1 (PC1), PC2 and furin were present in SK-N-MCIXC cells. PC1 and/or PC2 and/or furin may cleave at the dibasic amino acid pairs Arg-Arg at the C-terminal part of proCCK, and Arg-X-X-Arg at the N-terminal of the CCK-58 sequence in proCCK. The SK-N-MCIXC cell line demonstrated spontaneous and regulated release of CCK and large amounts of CCK-precursors, as measured with region specific radioimmunoassays coupled to high performance liquid chromatography. Storage granules containing glycine-extended CCK were shown in SK-N-MCIXC cells using indirect immunofluorescence. The extracellularly localized CCK-metabolizing enzyme, neutral endopeptidase 24.11 (EC 3.4.24.11), was present in membranes from both SK-N-MCIXC cells and in intact slices of rat cerebral cortex. The rat cerebral cortex is a brain region known to be rich in CCK. The SK-N-MCIXC cell line provides an in vitro model to study the regulation of CCK synthesis and metabolism in neuronal systems since it contains the storage granules, mRNA, intact peptide, and complement of enzymes necessary for biosynthesis and metabolism of CCK.

Amino Acid Sequence↗
PubMed1986

Translocation breakpoint mapping: molecular and cytogenetic studies of chromosome 22.

A number of clinically significant human diseases are associated with rearrangements of chromosome #22. These include the t(9;22) of chronic myelogenous leukemia (CML), the t(8;22) of Burkitt's lymphoma (BL), the t(11;22)(q23;q11) constitutional rearrangement and the t(11;22) of Ewing's sarcoma (ES) and neuroepithelioma (NE). All of these translocations have breakpoints in 22q11. Using a molecular cytogenetic approach and various cloned portions of the lambda light chain gene as probe, we have assigned a linear order within 22q11 to these breakpoints. Using chromosomal in situ hybridization we have determined that the 22q11 breakpoint in BL2, a t(8;22) Burkitt's lymphoma is proximal to the breakpoint of the t(9;22) of CML. We have demonstrated that the 22q11 breakpoint of PA682, another t(8;22) BL cell line, interrupts the lambda light chain locus. Using a combination of variable and constant region probes and PA682 cells, we have shown that the lambda light chain locus is oriented such that V lambda is proximal to C lambda in 22q11. Our results for the constitutional t(11;22) indicate that the 22q11 breakpoint is distal to that of BL, proximal both to that of CML and ES and, in addition, it interrupts the C lambda gene cluster. Our studies of the 22q11 breakpoint of the t(11;22) of ES and NE suggest that they are the most distal of the breakpoints we studied on chromosome #22.

Burkitt Lymphoma↗
PubMed1992

Translocation (5;22) in an Askin's tumor.

A boy with a brain metastasis of an Askin's tumor was investigated. Cytogenetic studies revealed a near-diploid karyotype with monosomies of chromosomes 5 and 22 in the presence of a derivative chromosome der(5)t(5;22)(q35;q11). In particular, no involvement of chromosome 11 was seen.

Brain Neoplasms↗