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)

The various stages of divergent neuroepithelial differentiation were studied in the solid transplants of a transplantable mouse testicular teratoma (OTT-6050) maintained in both ascitic and solid forms. They included: a) areas of undifferentiated medullary epithelium corresponding to the rare human medulloepithelioma; b) areas of neuroblastic differentiation corresponding to neuroblastoma, with more mature neuronal differentiation corresponding to ganglioneuroma or, when mixed with glial elements, to ganglioglioma; and c) more mature neuroglial areas resembling astrocytoma, oligodendroglioma or ependymoma, as well as more primitive areas corresponding to ependymoblastoma. In tissue culture using collagen-coated coverslips, astrocytic differentiation was found in the outgrowth zone after 15 days, confirmed by immunofluorescence with antibodies to an astroglia-specific protein. In organ culture systems, glial components, including ependymal structures, were preserved in tumor explants, and astrocytic differentiation, as expressed by glial fiber formation, was increased after 4 to 6 weeks in vitro. No neuronal differentiation was demonstrable, however. The neuroepithelial component of this experimental teratoma may provide a model for the study of neoplastic neuroepithelial differentiation.
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PMID:An experimental mouse testicular teratoma as a model for neuroepithelial neoplasia and differentiation. I. Light microscopic and tissue and organ culture observations. 16 76

A gas chromatographic method is described for the determination of urinary vanilglycolic acid, vanilglycol, vanilacetic acid and vanillactic acid as their trimethylsilyl derivatives. These metabolites are chemical parameters for the dignosis of neurogenic tumours. Their determination is especially recommended in order to evaluate the effect of the therapy. Results in normals and controls are given. Data in a number of selected patients with neuroblastoma, ganglioneuroma and phaeochromocytoma are presented and discussed.
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PMID:Gas chromatographic determination of urinary vanilglycolic acid, vanilglycol, vanilacetic acid and vanillactic acid-chemical parameters for the diagnosis of neurogenic tumours and the evaluation of their treatment. 18 88

Case reports of neuroblastoma revealed that some individuals are genetically predisposed and that this genetic predisposition may have other consequences. According to a mutation model, two classes of individuals could acquire neuroblastoma. One (prezygotic) was a rare class that carried a dominant gene imparting high risk of the tumor. The other (postzygotic) comprised all other individuals, each at low risk. The model related tumor incidence to germinal and somatic mutation rates and thereby carried implications for environmental modification of tumorigenesis and demographic variation in incidence. Case reports also revealed associations of neuroblastoma with congenital defects and a susceptibility to second tumors. Analogy with retinoblastoma and Wilms' tumor of the kidney suggested that these associations could result from action of a neuroblastoma gene or from chromosomal aberration. One known dominantly inherited condition, von Recklinghausen's disease, could dispose to neuroblastoma and create some associations. According to the two-mutation model, neuroblastoma may have been a single recessive gene disorder at the level of the cell. The phenomena of aganglionosis, neuroblastoma in situ, maturation of neuroblastoma to ganglioneuroma, and spontaneous regression suggested that such a neuroblastoma gene interfered with normal developmental processes. The specificities of this gene and of those for von Recklinghausen's disease and pheochromocytoma suggested that the functiof a membrane macromolecule.
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PMID:Developmental genetics of neuroblastoma. 18 2

Tissue content of cyclic AMP was as much as 10 times greater in ganglioneuroma than in neuroblastoma. This high cyclic AMP in ganglioneuroma was not significantly different from that of sympathetic ganglia.
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PMID:Cyclic AMP in neuroblastoma, ganglioneuroma and sympathetic ganglia. 20 Apr 62

The experience of one regional thoracic surgical unit in managing intrathoracic neural tumours over a 25-year period is presented. Neural tumour was diagnosed in 55 patients, of whom 41 were asymptomatic. In 11 patients complete resection was not achieved--the reasons for this and its effect on the outcome of the patient are discussed. There were 52 posterior mediastinal and three lateral chest wall tumours. The pathological distribution was as follows--benign nerve sheath tumours (neurofibroma, neurilemoma) 39, ganglioneuroma 13, and neuroblastoma 3. One neurofibroma recurred as a neurosarcoma six years after its apparently complete resection and was removed by an extensive resection at reoperation. One neuroblastoma recurred within the spinal canal four years after incomplete excision at thoracotomy--this patient died subsequently of widespread metastatic neuroblastoma. No other tumour is known to have recurred.
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PMID:Intrathoracic neural tumours. 21 May 31

Immunohistochemical studies have demonstrated that immunoreactive vasoactive intestinal peptide is present in, and restricted to, the differentiating and mature ganglion cells in a variety of normal and neoplastic neural tissues. In a composite pheochromocytoma-ganglioneuroma (associated with the syndrome of watery diarrhea, hypokalemia, and hypochlorhydria), five ganglioneuroblastomas, five ganglioneuromas (two of which were associated with diarrheal syndromes), an unusual mixed neuroblastoma-ganglioneuroma, and four normal sympathetic ganglia, vasoactive intestinal peptide was present in differentiating and mature ganglion cells. The peptide was also demonstrated in isolated ganglion cells in two pheochromocytomas but was not present in pheochromocytes, Schwann cells, or undifferentiated neuroblastic cells in the neuroblastomas and ganglioneuroblastomas. These studies indicate that the presence and presumably the production of vasoactive intestinal peptide thus reflect a particular line of neuroblastic differentiation and are not merely a reflection of common derivation of these tissues. Our identification of vasoactive intestinal peptide in neurogenic tumors associated with diarrhea supports the contention that the peptide might be an important diarrheogenic factor in these tumors.
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PMID:Vasoactive intestinal peptide and its relationship to ganglion cell differentiation in neuroblastic tumors. 22 58

A child with mediastinal and supraclavicular ganglioneuroma, heterochromia iridis, and Horner's syndrome is described. The authors postulate that the thoracic neoplasm originated as a congenital neuroblastoma with metastasis to the supraclavicular lymph nodes and subsequent benign transformation, and that the ocular abnormalities resulted from sympathetic ganglion injury by the tumor in infancy. This seems to be the first published report of such ocular abnormalities associated with mediastinal ganglioneuroma.
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PMID:Ganglioneuroma, heterochromia iridis, and Horner's syndrome. 51 8

Aberrations in the metabolic pathways of catecholamines in patients with neural crest tumors result in characteristic urinary excretion patterns of their catabolites. Tumors such as pheochromocytoma, neuroblastoma and ganglioneuroma usually defy clinical diagnosis because of their rarity, small size, intraabdominal position and clinical symptoms similar to those of essential hypertension. Quantitative determination of catecholamine metabolites such as vanillylmandelic acid (VMA) and 3-methoxy-4-hydroxyphenylethyleneglycol (MHPG) offers possibilities for reliable confirmation of diagnosis. However, previous techniques for the assessment of catabolite levels suffered from inadequate sensitivity, reproducibility or specificity, which seriously diminished their usefulness as biochemical determinants in the prognosis of these life-threatening tumors. Reported in this paper is the analysis of urinary levels of VMA and MHPG using reversed-phase high-performance liquid chromatography with electrochemical and sectrophotometric detection. We present the excretion patterns showing these metabolites in 15 control subjects, 15 patients with pheochromocytoma and 5 patients with neuroblastoma.
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PMID:Diagnosis of neural crest tumors by reversed-phase high-performance liquid chromatographic determination of urinary catecholamine metabolites. 54 40

A group of bioptically examined neuroectodermal tumours of the skin and soft tissues consisting, in particular, of 170 Schwannomas and 350 tumours has been reevaluated in a retrospective study. The following common classification of these tumours has been recommended: I. Tumours of Schwann's cells: 1. neurilemmoma [A and B], 2. neurofibroma [with the following variants v. Recklinghausen's type, plexiform, pigmented, Paccinian, with Meissner-Wagner's bodies and meningiomatous], 3. amputation neuroma, 4. neurosarcoma, 5. others. II. Melanogenic tumours: A. pigmented naevi (junction, mixed, intradermal, epithelioid, clear cell, halo, neurocutaneous, fibrous, blue, proliferating blue, melanotic progonoma, others). -B. praecancerous melanosis. -C. malignant melanoblastoma (common type, from praecancerosis). III. Tumours of ganglion cells: 1. ganglioneuroma, 2. neuroblastoma, 3. paragangliomas (with granules, without granules, alveolar soft part sarcoma).
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PMID:[Neuroectodermal tumors of the skin (a normative study)]. 59 24

160 tumors of neural origin occurring in the thorax were analyzed. The major histological features of schwannoma, neurofibroma, neuroblastoma, ganglioneuroblastoma, ganglioneuroma, and paraganglioma are described. Radiological analysis emphasized shape and location. Calcification was relatively uncommon but may be specific. The comparatively low figures on incidence of rib and vertebral abnormalities might be increased by special studies, including vertebral tomography. Evidence of local spread such as pleura-based nodules and pleural effusion constitutes evidence of malignancy. Age may be the most important clinical parameter for distinguishing between histological types.
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PMID:Neural tumors of the thorax: subject review from the AFIP. 61 41


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