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)

Twenty-four patients with the histological diagnosis of olfactory neuroblastoma have been treated at the Royal National Throat, Nose and Ear Hospital since 1975. The tumour showed a bimodal age distribution and 30% of the patients were under 30 years of age. The imaging characteristics on plain film, computed tomography and magnetic resonance combined with intravenous gadolinium DTPA are detailed. None of the changes described is wholly specific. However, a tumour in the ethmoids and upper part of the nasal cavity, which expands into the orbit and erodes the roof of the fronto-ethmoid complex or cribriform plate unilaterally in a young patient, is highly suggestive of olfactory neuroblastoma, particularly if this is combined with the magnetic resonance signal characteristics of a vascular tumour. The typical MR features are those of an intense signal on pre-contrast T2 weighted spin echo sequences and strong enhancement after gadolinium on T1 weighted sequences. A characteristic feature of the response to gadolinium is an enhancement of tumour higher than that of turbinate mucosa on inversion recovery and less than that of mucosa when T1 weighted spin echo sequences are employed. The extent of tumour in the paranasal sinuses and anterior cranial fossa is best demonstrated after magnetic resonance with intravenous gadolinium and this is now regarded as the most accurate method of preoperative assessment of these patients prior to craniofacial surgery.
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PMID:Olfactory neuroblastoma: imaging by magnetic resonance, CT and conventional techniques. 324 16

This paper describes the immunohistochemical staining properties of four monoclonal antibodies (MAbs) (CF, EB, AD, and KB) which had been previously shown to be specific for purified neuron-specific enolase (NSE) by a solid-phase radioimmunoassay. In this study, the authors immunostained a spectrum of normal and neoplastic neuronal, "neuroendocrine," and nonneuronal tissues fixed in formalin and embedded in paraffin. Positivity was generally restricted to normal neuronal structures and neuronal tumors, including adrenal neuroblastoma, ganglioneuroblastoma, olfactory neuroblastoma, pheochromocytoma, carotid body paraganglioma, duodenal gangliocytic paraganglioma, and teratoma with neuroepithelial components. Three staining patterns of the normal or neoplastic neuronal structures were observed: two MAbs (CF and EB) stained predominantly the nerve fibers (axoplasm); one (AD) stained predominantly the cell bodies (perikaryon); and one (KB) stained both the axoplasm and the perikaryon. "Neuroendocrine" tumors such as pulmonary small cell carcinoma, pancreatic islet cell tumor, thyroid medullary carcinoma, and carcinoid tumors from various locations showed a variable staining pattern. Tumor cells undergoing mitotic division were usually positive regardless of type. Normal structures other than neuronal or "neuroendocrine," including normal glial cells, were negative. The authors also studied a range of glial cell tumors with MAbs CF and AD as well as with Dako polyclonal antiserum to NSE. The results showed that CF stained the axonal fibers in the normal white matter surrounding these tumors; it did not stain the tumor cells or the perikarya of neurons in the surrounding normal gray matter. AD stained the glioma cells as well as the perikarya and dendrites of neurons in the surrounding normal gray matter; it did not stain the axonal fibers in the surrounding normal white matter. By contrast, the polyclonal antiserum stained all of these structures. The high degree of staining specificity of the MAbs should prove them to be valuable in immunohistochemical diagnosis of tumors as well as in further understanding the role of NSE in neuronal differentiation.
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PMID:Immunohistochemical characterization of a set of monoclonal antibodies to human neuron-specific enolase. 328 44

Esthesioneuroblastoma (olfactory neuroblastoma) is a rare malignant neoplasm derived from the olfactory epithelium. It was diagnosed by cytologic study of fine needle aspiration biopsy smears of a tumor situated in the nasopharynx in a 57-year-old man.
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PMID:Cytologic appearance of esthesioneuroblastoma in a fine needle aspirate. 337 5

The efficacy of two new monoclonal antibodies with cell lineage-restricted reactivity (HMB-45 [melanocytes] and anti-synaptophysin [neuroepithelial cells]) was compared with that of "traditional" antibody panels in the delineation of malignant melanoma (MM) of the sinonasal region, nasopharyngeal carcinoma (NPC), and olfactory neuroblastoma (ONBL). HMB-45 recognized all of eight melanomas and stained one of five neuroblastomas, but failed to label any of 12 cases of NPC. All examples of ONBL were stained by anti-synaptophysin; other tumors were nonreactive with this reagent. A panel of antibodies to cytokeratin, vimentin, epithelial membrane antigen, and S100 protein was also effective in discriminating between MM, NPC and ONBL. These results suggest that HMB-45 and anti-synaptophysin are comparable in utility to more extended antibody panels in the diagnosis of sinonasal malignancies, but only if used in combination with one another.
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PMID:Immunohistochemical diagnosis of sinonasal melanoma, carcinoma, and neuroblastoma with monoclonal antibodies HMB-45 and anti-synaptophysin. 337 60

In these studies, strong HLA-A,B,C or b2-m expression was not detected in any stage of development of normal neurons. These included regenerating cells of the olfactory epithelium, developing neural tissue in the mouse embryo, and normal adult brain. Nor was an obligatory role for HLA-A,B,C or b2-m revealed by in vitro analysis of a growing neuroblastoma cell line.
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PMID:Biological significance of HLA-A,B,C expression in neuroblastoma and related cell lines. 340 9

An immuno-electron microscopic study performed by a pre-embedding peroxidase-antiperoxidase (PAP) technique revealed S-100 protein reactivity in the cytoplasm and connecting processes of marginal cells of olfactory neuroblastomas. The overlapping cytoplasmic processes of S-100 immunoreactive cells completely surrounded the main membrane-bound granule-bearing tumor cells forming a continuous interface between those tumor cells and fibrous connective tissue. The S-100 immunoreactivity was also observed in the cytoplasm and processes on non-membrane-bound granule-bearing tumor cells within the tumor nodule. The tumor cell nodules and isolated tumor cells were completely surrounded by basement membrane material stainable with anti-laminin antiserum. It appears that the membrane-bound granule-bearing tumor cells of olfactory neuroblastoma are partitioned by basement membrane of S-100 protein-reactive cells and their processes. No S-100 protein-reactive cells were observed within the olfactory epithelium of the two patients with olfactory neuroblastoma. To determine a possible origin of S-100 protein-positive cells, normal olfactory epithelium from six adult patients at autopsy was studied for S-100 reactivity. There were no S-100 protein-positive cells within the normal olfactory epithelium. We discuss the evidence for considering olfactory neuroblastoma a variant of paraganglioma.
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PMID:Olfactory neuroblastoma: an immuno-electron microscopic study of S-100 protein-positive cells. 352 97

A case of olfactory neuroblastoma metastatic to the choroid is reported and described. Histologically, the tumor consisted of islands of small round cells, many of them surrounding blood vessels in a 'pseudorosette' pattern. Numerous mitotic figures were present. Transmission electron microscopy reveals neuritic processes containing neurosecretory granules and microtubules, features characteristic of olfactory neuroblastoma. This is the first published case of an olfactory neuroblastoma demonstrating intraocular metastasis.
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PMID:Olfactory neuroblastoma metastatic to the eye. 365 24

Olfactory neuroblastoma is a rare tumor arising from the olfactory mucosal epithelium. 5 patients with this tumor were treated in our hospital from 1978 to 1982. The youngest patient was 3 years old and the eldest was 66. The clinical symptoms were nasal stuffiness, rhinorrhea, epistaxis and vascular polypoid mass in the nasal cavity. According to the staging system proposed by Kadish et al, the five patients in this series were 3 stage A and 2 stage C patients. Treatment consisted of radiotherapy, surgery or combination of radiotherapy and surgery. Our data indicate that the olfactory neuroblastoma is a radiosensitive tumor with the prognosis favorable in the stages A and B patients as treated by these three modalities. Two stage A patients are alive for more than 5 years. One of them was treated by radiotherapy alone, the other by combination of surgery and radiation. The third stage A patient as treated by radiotherapy alone has survived more than 3 years. One stage C patient, treated by combination of radiation and surgery, is still alive for more than 3 years. The other stage C patient, treated by radiotherapy alone, had survived for only 7 months after the treatment. The radiation dose varies with the extent of invasion. In stages A and B lesions, a dose of 4,500-5,500 rad in 5 weeks may be reasonable but in stage C, a dose of 6,000-6,500 rad in 7 weeks should be given. The authors agree to the prognostic equation proposed by Homzie et al. It may be possible to predict the tumor control or recurrence after a period of 3 years which gives an accuracy rate of 87%.
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PMID:[Radiotherapy of olfactory neuroblastoma--report of 5 patients]. 374 53

A 28-year-old man with the chronic syndrome of Inappropriate antidiuretic hormone secretion and hypertension was found to have an olfactory neuroblastoma. We demonstrated evidence of elevated circulating arginine vasopressin levels, significantly elevated arginine vasopressin and vasopressin neurophysin levels in the tumor extract, and immunohistochemical staining for arginine vasopressin and vasopressin neurophysin in the tumor cells. The patient's clinical syndrome, including hypertension, resolved following subtotal removal of the tumor and radiation therapy. This study identified olfactory neuroblastoma as a definite cause of ectopic arginine vasopressin secretion causing the syndrome of inappropriate antidiuretic hormone secretion.
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PMID:Chronic syndrome of inappropriate antidiuretic hormone secretion and hypertension in a patient with olfactory neuroblastoma. Evidence of ectopic production of arginine vasopressin by the tumor. 375 13

A 26-year-old woman with the syndrome of inappropriate antidiuresis demonstrated complete recovery following the resection of an olfactory neuroblastoma. Tissue arginine vasopressin levels by radioimmunoassay, immunohistochemical staining of the tissue arginine vasopressin, postoperative normalization of plasma arginine vasopressin levels, and the clinical resolution are evidence in support of a neurally derived tumor being the direct source of neurosecretion of arginine vasopressin rather than neurohypophyseal secretion or secretion from non-neural tissues, as reported to date in the etiology of the syndrome of inappropriate antidiuresis.
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PMID:Neurosecretion of arginine vasopressin by an olfactory neuroblastoma causing reversible syndrome of antidiuresis. 377 95


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