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)

We report a retrospective study of two years experience with I-131-metaiodobenzylguanidine (MIBG). I-131 MIBG was prepared locally and was found to have decreased background activity as compared with other available commercial preparations from Great Britain and the United States. Fifty-nine patients were studied with a total of 65 scans. The study included 11 members of a family with multiple endocrine adenomatosis (MEA) type II syndrome. Of 16 patients found to have abnormal scans, 12 had disease confirmed surgically. These cases consisted of nine pheochromocytomas, one paraganglioma, one neurilemmoma, and one neuroblastoma. MIBG scans for pheochromocytoma detection had an accuracy of 94.5%, a sensitivity of 100%, and a specificity of 93.5% in this study of patients with a high prior probability of the disease.
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PMID:Experience with I-131-metaiodobenzylguanidine (MIBG): a retrospective study. 288 22

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

Neurogenic tumors of the thorax are observed in all age groups, although they are rare in the elderly. They are more likely to be malignant in the child than in the adult. Tumors of the autonomic system are common in children, whereas the nerve sheath tumors are more likely to be found in adults. The malignant lesions are almost always symptomatic and the benign lesions asymptomatic, except in the child, in whom a benign lesion may result in symptoms because of its size relative to the volume of the child's thorax. Intraspinal canal extension, although relatively infrequent, should be sought for in all paravertebral tumors, for a significant percentage of these may be initially asymptomatic. Excision of such an hourglass tumor without foreknowledge of such extension may lead to serious spinal cord complications. Surgical excision of most of these tumors is sufficient except for the malignant lesions, especially in infancy and childhood, for which the addition of postoperative irradiation and chemotherapy may be beneficial. The prognosis after the removal of benign neurogenic tumors of the thorax is excellent. In the infant or child with Stage III or IV neuroblastoma, ganglioneuroblastoma, or an Askin tumor, the prognosis is poor. In the adult with a malignant neurogenic sarcoma or a malignant paraganglioma, the prognosis likewise is grave.
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PMID:Neurogenic tumors of the thorax. 337 59

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

Critical Evaluation of 200 tumours of meninges, brain and spinal cord showed that to be familiar with the ultrastructural features of meningioma and its variants was instrumental in differential diagnosis of other primary or secondary meningeal tumours (neurinoma, paraganglioma, xanthomatous and histiocytic tumours). A limited value of electron microscopy was found in astrocytoma and glioblastoma in contrast to its importance in low-differentiated ependymoma and oligodendroglioma. The examination had histogenetical and taxonomic values in medulloblastoma (CNS neuroblastoma and mixed tumours with a component featuring primitive neuroectodermal or neuroblastic differentiation). Ultrastructure was very important in the so-called primitive neuroectodermal CNS tumours where only the lack of conspicuous glial or neuroblastic differentiation confirmed the diagnosis. Electron microscopy was instrumental in rare primary CNS lymphomas as well as in some metastatic tumours.
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PMID:[Contribution of electron microscopy in the differential diagnosis of tumors of the meninges, brain and spinal cord]. 373 Dec 97

Monoclonal antibodies that recognize either neurofilaments or glial filaments were used with the peroxidase-antiperoxidase (PAP) method to retrospectively study 100 tumors of the central and peripheral nervous systems in paraffin-embedded sections. Only neoplasms of putative neuronal origin or with presumed neuronal differentiation (paraganglioma, ganglioglioma, ganglioneuroblastoma, ganglioneuroma, neuroblastoma, ovarian teratoma and pheochromocytoma) contained tumor cells with immunoreactive neurofilament, but such cells were more common in the more differentiated or benign neoplasms in this category. Glial filament immunoreactivity was observed in tumor cells of glial origin and in tumor cells with foci of glial differentiation arising within the central nervous system, consistent with findings from previous studies using anti-glial-filament antisera. With the exception of a benign cystic teratoma, no glial filament immunoreactivity was observed outside the central nervous system. Some immunoreactive neurofilaments, but not glial filaments, were arranged in presumably abnormal balls, cords, or clumps within tumor cells, possibly reflecting cytoskeletal alterations related to neoplastic transformation. These findings indicate that monoclonal antibodies against intermediate filament proteins such as neurofilaments and glial filaments retain their specificity and sensitivity when employed in paraffin sections in conjunction with the peroxidase-antiperoxidase method. They suggest that such reagents are useful probes for the evaluation of the histogenesis or degree of differentiation in human nervous system tumors. Finally, they permit the speculation that the analysis of the intermediate filaments of tumor cells, as contrasted with those in normal cells, may provide new insights into the biology of neoplasms.
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PMID:An immunohistochemical study of human central and peripheral nervous system tumors, using monoclonal antibodies against neurofilaments and glial filaments. 653 79

In normal conditions, neuron-specific enolase (NSE) is histochemically demonstrable only in neurons and cells of the amine precursor uptake and decarboxylation (APUD) system. This has been found not to be true for neoplastic cells. Several types of CNS tumors, including glioblastoma, astrocytoma, oligodendroglioma, ependymoma, medulloblastoma, pineocytoma , meningioma, and choroid plexus papilloma, focally stained positively for NSE. Reactive astrocytes were also frequently positive. In the peripheral nervous system, neuroblastoma, ganglioneuroma, and paraganglioma stained positively for NSE. A number of non-APUD tumors were focally positive. These included schwannoma, carcinoma and fibroadenoma of the breast, renal cell carcinoma, giant cell tumor of the tendon sheath, and chordoma. Caution should be exercised in relying on the immunohistochemical demonstration of NSE as a diagnostic marker in those tumors that do not belong to the APUD cell system. It seems of little value as evidence of differentiation in CNS tumors.
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PMID:Immunohistochemical demonstration of neuron-specific enolase in neoplasms of the CNS and other tissues. 654 18

Between 1957 and 1978 the authors operated 15 patients with an adrenal tumor. This series includes six pheochromocytomas, seven cortical tumors, one adrenal cyst and one neuroblastoma. The specific diagnostic and therapeutic problems encountered in this group of different type tumors are discussed. In the group of six pheochromocytomas one was benign but recurred nine years later, one was a paraganglioma and one a malignant pheochromocytoma with functional glandular metastases. The seven cortical tumors are divided into one functional benign tumor, two non-functional benign tumors of which one was located outside the adrenal gland, two non-functional malignant tumors, one functional malignant tumor and one syndrome of Conn.
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PMID:[Surgical experiences with adrenal tumors (author's transl)]. 724 96

The intracellular localization of tyrosine hydroxylase (TH), which is the rate limiting enzyme in catecholamine (CA) biosynthesis, and its activity in various adrenal and other neuroendocrine tumors was studied. TH was strongly localized in adrenal medulla, pheochromocytoma, and paraganglioma, but was scatteredly expressed in neuroblastoma. TH was not detected in adrenocortical tumors, ganglioneuroma, and other neuroendocrine tumors. Neuron specific enolase (NSE) was found in all neuroendocrine tumors, but Grimelius staining showed only the secreting granules of the tumor cells. TH activity was significantly high in pheochromocytoma and paraganglioma as compared with that in normal adrenal gland, whereas TH activity was low in a neuroblastoma and was undetectable in other tumors. These findings indicate that TH correlates well with the biosynthetic function of CA in the tumor cell and, thus, both the immunostaining of TH and the measurement of its activity in adreno-medullary and related tumors may provide some information about the process of cell differentiation in these tumors.
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PMID:Tyrosine hydroxylase indicates cell differentiation of catecholamine biosynthesis in neuroendocrine tumors. 752 77

In the I-131 MIBG scans of 14 patients with neuroblastoma (86%) or pheochromocytoma/paraganglioma (14%) that were studied more than 48 hours after administration of the radiopharmaceutical, 12 (86%) had discernible cerebellar MIBG localization. A few had midbrain or diffuse cerebral uptake as well. None of the patients had cerebellar or other central nervous system signs or symptoms, and the localization is consistent with the known distribution density of central nervous system catecholamine receptors. This suggests that cerebellar MIBG localization is normal in delayed scans and that it should not be confused with neuraxial metastasis of adrenergic neuronal neoplasms.
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PMID:Normal cerebellar MIBG localization. Implications in the interpretation of delayed scans. 784 95


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