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)

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

Selected examples of neoplasms of the central nervous system are reviewed in which the electron microscope has been instrumental in establishing the diagnosis. These include ependymoma, ependymoblastoma, epithelial cyst, paraganglioma in the cauda equina and cerebellar neuroblastoma.
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PMID:Some contributions of electron microscopy to the diagnosis of brain tumors. 67 76

Presentation of a series of 14 cases of neural crest derived tumours located in the retroperitoneal space in adult patients (five pheochromocytoma, six paraganglioma, two ganglioneuroma, and one neuroblastoma), and review and update of the diagnostic and therapeutic aspects. All pheochromocytoma cases presented high BP and the classic triad of sudation, tachycardia and headaches, as well as high levels of blood and urine catecholamines and/or their metabolites. CAT, ultrasound scanning and 123MIBG were the main diagnostic techniques used. All four paraganglioma were functioning and generally located surrounding both kidneys (one case was paired). No malignancy was found in any of the 11 tumours while controls remain with normal BP and normal levels of urine catecholamine metabolites. None of the two ganglioneuromas showed specific signs and symptoms but were diagnosed accidentally. The one neuroblastoma was juxtavesical showing a highly unfavourable evolution in spite of radical surgery, radiotherapy and multiple chemotherapy and the patient died within 16 months with local recurrence and haematogenous dissemination to bones and lungs.
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PMID:[Neural crest derived retroperitoneal tumors. General review]. 131 88

Calcineurin is a calcium/calmodulin-regulated protein phosphatase. By using enzyme-immunoassay and immunocytochemistry with an affinity-purified specific antibody to this protein, we have found that calcineurin is expressed in the central and peripheral neuroendocrine cells, also termed amine precursor uptake and decarboxylation cells. In addition, calcineurin immunoreactivity was found in the central neuroendocrine neoplasms such as pineocytoma, olfactory neuroblastoma and paraganglioma. The present findings indicate that the activity of phosphatase regulated by calcium and calmodulin is involved in neuroendocrine functions, and that the enzyme can be useful for the identification and characterization of neuroendocrine cell tumors.
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PMID:Calcineurin, a calcium/calmodulin-regulated protein phosphatase, in mammalian neuroendocrine cells and neoplasms. 133 5

From 1967 to 1991 we have diagnosed and treated 73 adrenal tumors in 63 patients: 12 pheochromocytomas, 24 adrenal cortical adenomas, 15 hyperplasias, 16 carcinomas, 3 myelolipomas, 2 cysts and 1 neuroblastoma. We conducted a retrospective study to analyze the preoperative images obtained by different diagnostic techniques and attempted to correlate tumor size and site with the results of the histological analysis of the surgical specimen. Nephrotomography with pneumoretroperitoneum and IV Nephrotomography were useful in detecting the increase of the size of the gland in 10 of 25 cases submitted to these procedures (40%). Arteriography as second or third technique of choice confirmed the presence of an adrenal tumor in 15 of the 21 cases evaluated by this procedure (70%). US and CT detected 94% (31/33) and 100% (33/33) of the cases, respectively. Fourteen cases were incidentally discovered by CT (7) and US (7). A direct relationship between tumor size and degree of malignancy could be established since the carcinomas had a mean diameter of 7 cm (range 5 to 12 cm). Concerning the histologic nature of the disease, specific images were found in 3 cases of adrenal myelolipoma (hyperechoic on US and of low density similar to fat on CT) and 2 cysts (anechoic with posterior band evidenced on us and liquid on CT). Radioisotopes were also utilized for tumor localization and there was positive uptake of I-131-IMBG in 2 cases of adrenal pheochromocytoma; 1 extra-adrenal (left lateral aortic paraganglioma) and 1 case of malignant adrenal pheochromocytoma with metastasis to the lungs.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[The imaging diagnosis of adrenal tumors]. 160 87

Immunohistochemical studies have shown that almost all thymomas of myasthenia gravis patients contain at least one protein sharing an antigenic determinant with the nicotinic acetylcholine receptor (AchR) of human muscle. We describe the characterization of this protein (p153) which has a molecular weight of 153 and an isoelectric point of 5.0. By treatment of p153 with endoglycosidases, no significant glycosylation has been detected. Immunologically, p153 crossreacts with monoclonal antibodies against the amino acid sequence 371-378 of the alpha-chain of the AchR. No cross-reactivity to the main immunogenic region of the AchR nor an alpha-bungarotoxin binding site are found. By Western blotting, p153 was generally neither detectable in normal tissues nor extrathymic tumors with the exception of paraganglioma and neuroblastoma. In conclusion, the structure of p153 is apparently unrelated to the AchR from muscle or the alpha-bungarotoxin binding proteins from thymoma. Since there is no evidence for an AchR expression in thymoma, the antigenic homology of p153 with the nicotinic AchR might be relevant for triggering an intrathymomatous autosensitization of maturing T cells and could be responsible for the high association of thymomas with myasthenia gravis.
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PMID:Characterization of a protein with an acetylcholine receptor epitope from myasthenia gravis-associated thymomas. 215 8

In recent years, *I-MIBG (*I-metaiodobenzylguanidine), which is transported and stored in the chromaffin cells, has been shown to allow good visualization of neuroblastomas in children. This paper deals with 30 *I-MIBG-scans performed in 20 children: 16 with neuroblastoma, 3 with retinoblastoma, and 1 with a malignant paraganglioma. A high detection rate was found for both primary and secondary sites of neuroblastoma. *I-MIBG was generally superior to 99mTc-MDP bone scintigraphy in the detection of bone metastases. Our experience illustrates the unique place of *I-MIBG-scintigraphy compared with other imaging techniques: it makes it possible to define the nature of the tumour, particularly in cases with normal catecholamine levels; to establish how extensive the lesions are at the time of diagnosis; and to confirm complete remission. No abnormal *I-MIBG uptake was noted in the 3 cases of retinoblastoma.
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PMID:Consolidating the role of *I-MIBG-scintigraphy in childhood neuroblastoma: five years of clinical experience. 235 93

Identification of growth factors and receptors in mesenchymal tumors may be crucial to understanding of growth regulation in sarcomas. During an immunohistochemical study of the expression of growth factors and receptors in human soft tissue tumors (STT), only 1 antisera capable of working in paraffin-embedded tissue was noted. A detailed study of 141 STT was undertaken to determine the frequency of expression of nerve growth factor receptor (NGF-R), its specificity and sensitivity for neural tumors, and the effect of fixation on detection. In normal mesenchymal tissue, only nerve sheath and perivascular staining was seen. No immunoreactivity was seen in many tumors including rhabdomyosarcoma, angiosarcoma, liposarcoma, Ewing's sarcoma, and alveolar soft part sarcoma. Less than 15% of tumors of smooth muscle, fibrous, or fibrohistiocytic origin showed immunoreactivity, usually focal. In contrast, a high frequency of immunoreactivity was noted in tumors of neural origin (74%). This included granular cell tumors (100%), Schwannoma/neurofibroma (91%), malignant Schwannoma (78%), neuroblastoma/neuroepithelioma (60%), and paraganglioma (57%). A high rate of reactivity was also seen in synovial sarcomas (80%), undifferentiated sarcomas (60%), and hemangiopericytomas (43%), suggesting a potential relationship to the neural phenotype. Among the neural tumors, Bouin's fixation was superior to formalin, suggesting that immunoreactivity for NGF-R is affected by fixation. This antibody may be a useful adjunct marker diagnostically.
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PMID:Expression of nerve growth factor receptor in paraffin-embedded soft tissue tumors. 245 20

Nonchromaffin paraganglioma (NCP), also called glomus body tumor or chemodectoma, is rarely found in the orbit. The behavior of orbital nonchromaffin paraganglioma may potentially be more aggressive than in other head and neck locations. Diagnosis depends on electron microscopic demonstration of membrane-bound neurosecretory granules. Results of histopathologic study show a well-circumscribed lesion without a true capsule with alveolar or organoid arrangements of epithelioid cells within a reticulin framework with thin-walled blood vessels. Cells are polygonal with round or oval nuclei containing rare mitotic figures and pale-staining cytoplasm. Differential diagnosis includes alveolar soft-part sarcoma, alveolar rhabdomyosarcoma, neuroblastoma, carcinoid, and granular cell tumor. Of 29 previously reported cases of orbital NCP, 16 have been reclassified as alveolar soft-part sarcoma. The authors report a patient with an electron microscopically established orbital NCP, with the history of a contralateral glomus jugulare tumor irradiated 14 years previously.
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PMID:Orbital nonchromaffin paraganglioma. A case report and review of the literature. 255 86

An autopsied case of a malignant paraganglioma of the posterior thoracic cavity is reported. A 68-year-old man had complained of chest discomfort, and serial examinations revealed a functioning paraganglioma with bone metastasis. After death a pathological examination revealed that the tumors consisted of alveolarly arranged cells and well developed capillary vessels. Numerous neurosecretory granules were observed on viewing by electron microscopy. An immunohistochemical examination showed that most of the tumor cells were positive for NSE, while only a few cells were positive for the S-100 protein. These results indicate that a paraganglioma originating from the aortic sympathetic paraganglia had similar features of a carcinoid and a neuroblastoma.
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PMID:[An autopsied case of malignant paraganglioma of the posterior thoracic cavity]. 271 85


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