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)

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

To study the properties of protein-bound oligosaccharides in neuronally differentiating cells, two model systems were used: murine N1E-115 and N-18 neuroblastoma cells inducible by serum starvation and rat PC12 pheochromocytoma cells inducible by nerve growth factor. Glycopeptides were prepared from cells metabolically labeled with [3H]glucosamine and analyzed by gel filtration. The properties of the high-molecular-weight glycopeptides were studied using enzymatic digestion with neuraminidase and endo-beta-galactosidase. In contrast to other cell lines analyzed, the neuroblastoma and pheochromocytoma lines contained predominantly glycopeptides completely cleavable with endo-beta-galactosidase, which indicated that they were linear-type poly-N-acetyllactosamine glycans. The proportion of these linear chains in the high-molecular-weight fraction increased during neuronal differentiation in both cell systems. The linear nature of the glycans was also correlated with positive anti-i and negative anti-I reactivity of the cells in immunofluorescence microscopy. Specific cell surface labeling for poly-N-acetyllactosamine glycans and sodium dodecyl sulfate-polyacrylamide gel electrophoresis revealed several glycoprotein components, some of which showed changes during neuronal differentiation. The high proportion of linear poly-N-acetyllactosamine chains in these neuronal cell lines and its increase during neuronal differentiation suggests that these glycans may be a characteristic feature of neuronal or neuronally differentiating cells.
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PMID:Poly-N-acetyllactosamine glycans of cellular glycoproteins: predominance of linear chains in mouse neuroblastoma and rat pheochromocytoma cell lines. 330 6

The ability of a T1-weighted spin-echo magnetic resonance (MR) sequence to allow differentiation of benign from malignant adrenal masses at 0.5 T was investigated in 28 patients with 35 adrenal masses. All nine lesions with an adrenal mass-liver signal intensity ratio of 0.71 or less were metastases, and all 15 with a ratio of 0.78 or more were adenomas. Eleven masses (31%)--including six adenomas, three metastases, a pheochromocytoma, and a neuroblastoma--had ratios between these values. Nine of ten masses with adrenal mass-fat intensity ratios of 0.35 or less were metastases, and all 12 with ratios of 0.42 or more were benign. Eleven masses (31%), four malignant and one benign, had ratios between these values. The ratios for two masses could not be calculated due to lack of fat. The specificity of T1-weighted MR imaging in differentiating benign from malignant adrenal masses appears similar to that reported for T2-weighted imaging. However, significant overlap occurred, as has also been reported for T2-weighted imaging. While both imaging sequences may help distinguish benign from malignant adrenal masses in some cases, biopsy is still necessary when an accurate histologic diagnosis is essential.
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PMID:Adrenal masses: characterization with T1-weighted MR imaging. 333 11

We used a radioenzymatic method to determine plasma levels of free and total (free plus sulfoconjugated) norepinephrine and dopamine in 20 children with neuroblastoma (two were hypertensive), seven patients with pheochromocytoma, and 39 normotensive controls (24 children and 15 adults). No significant differences were noted between the two control groups. Patients with neuroblastoma and pheochromocytoma showed significantly higher levels of free and total norepinephrine than controls (p less than 0.01), and those with pheochromocytoma had higher levels than those with neuroblastoma (p less than 0.01). Although the differences were not statistically significant, free dopamine was higher in both groups of patients than in controls. Total dopamine was significantly higher in patients than in controls (p less than 0.01). A positive correlation was noted between levels of total norepinephrine and total dopamine in controls (r = 0.41, p less than 0.05) and in patients with neuroblastoma (r = 0.72, p less than 0.001). Such a correlation was not found in patients with pheochromocytoma. The total dopamine/total norepinephrine ratio was higher (p less than 0.005) in patients with neuroblastoma than in controls and patients with pheochromocytoma. Patients with pheochromocytoma had significantly lower ratios than the other groups (p less than 0.001). A negative correlation was found between the ratios in the different groups and either systolic (p less than 0.001) or diastolic (p less than 0.001) blood pressure. Our results not only support a role for plasma dopamine in the regulation of blood pressure but also suggest that, regardless of the actual levels of both catecholamines, a balance has to be achieved in order to maintain normal blood pressure levels.
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PMID:Total plasma dopamine/norepinephrine ratio in catecholamine-secreting tumors. Its relation to hypertension. 334 58

131I MIBG is taken up and stored by neural crest tumors, essentially pheochromocytoma and neuroblastoma. MIBG diagnosis in neuroblastoma has been attempted with the following results. 205 total body scintigrams were performed in 60 patients with neuroblastoma with doses of MIBG ranging from 0.5 to 1 mCi. 52 were positive, 6 in complete remission were negative, and 2 were false negatives in adults with tumors showing no secretion of metabolites. More than 90% of neuroblastoma are MIBG positive, and therefore MIBG imaging is now considered the most valuable means of diagnosis and staging of these tumors. 131I MIBG therapy has been attempted in 22 patients with neuroblastoma. They received multiple therapeutic doses of 41 to 2,090 mCi given IV at 3- to 6-week intervals. The results were 5 complete remissions, 10 partial remissions, 1 no change, 2 progressive disease and 1 lost to FU. Apart from bone marrow depression in patients with previous bone marrow involvement, the treatment was well tolerated. Six adults with other neural crest tumors were also treated. Pain relief in metastatic patients is a common and important result of MIBG therapy.
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PMID:131I-meta-iodobenzylguanidine in diagnosis and treatment of neuroblastoma. 335 53

We report a case of the rare concurrence of total colonic aganglionosis and central hypoventilation. The relevant literature regarding the association of these syndromes, the reversal of male to female predominance in total colonic aganglionosis v Hirschsprung's disease, their association with pheochromocytoma and neuroblastoma, and the possible etiologic role of cessation of neural crest cell migration, are discussed.
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PMID:Total colonic aganglionosis (Zuelzer-Wilson syndrome) and congenital failure of automatic control of ventilation (Ondine's curse). 343 Mar 2

Iodine-131 metaiodobenzylguanidine (131I-MIBG) is concentrated in a variety of neuroendocrine tumors, such as pheochromocytoma and neuroblastoma. Other neuroendocrine tumors from the APUD-cell system such as carcinoid tumors, may possess this uptake capability as well. We investigated 11 patients suffering from intestinal carcinoid with 131I-MIBG in order to determine the value of MIBG scintigraphy in these tumors. MIBG scans were positive in 5 out of 11 patients (45%). False-positive MIBG-scans did not occur. No correlation between MIBG uptake, clinical symptoms and urinary 5-HIAA level could be found.
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PMID:[Role of 131I-metaiodobenzylguanidine scintigraphy in the diagnosis of intestinal carcinoids]. 343 70

We constructed a human pheochromocytoma cDNA library and used differential hybridization to human pheochromocytoma and human neuroblastoma cDNA probes to isolate genes that are highly expressed in the adrenal medullary neuroendocrine tumor, pheochromocytoma, but not in the more immature embryonal tumor of adrenal medulla, neuroblastoma. Two cDNA clones, pG8 and pG2, were more highly expressed in normal and neoplastic chromaffin tissue than they are in neuroblastoma. Furthermore, they are expressed in a remarkably limited number of other human tumors or normal tissues. pG8 is highly expressed in medullary thyroid carcinoma, another tumor of neural crest origin, which can occur in association with pheochromocytoma in the multiple endocrine neoplasia type II syndrome. pG2 is highly expressed in the adrenal cortex, an endocrine gland thought to be embryologically unrelated to the neural crest-derived adrenal medulla. The expression of both pG8 and pG2 can be induced in human neuroblastoma cells with dexamethasone, suggesting a mechanism by which glucocorticoids may influence development of a neuroendocrine phenotype.
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PMID:Molecular markers of neuroendocrine development and evidence of environmental regulation. 347 Jul 97

Cell lines established from small cell lung cancer (SCLC), a neuroendocrine tumor, have low or absent expression of class I major histocompatibility complex antigens. To determine whether this phenomenon occurs also in vivo, 244 routine paraffin-embedded tumors including 32 SCLC and 79 non-SCLC (NSCLC) lung cancers were studied for expression of beta 2-microglobulin (beta 2m) by an avidin-biotin coupled immunoperoxidase technique. The majority of SCLC tumors lacked beta 2m expression, while some had weak, focal expression. In contrast, most NSCLC expressed beta 2m, often strongly. The difference between SCLC and NSCLC was highly significant statistically, suggesting that beta 2m can be used as a clinical immunodiagnostic marker for distinguishing NSCLC from SCLC. In addition, certain other neuroendocrine tumors (neuroblastoma, bronchial and midgut carcinoid tumors) lacked beta 2m expression, whereas some (pheochromocytoma, medullary thyroid carcinoma, and peripheral neuroectodermal tumors) usually stained positively. Such non-neuroendocrine tumors as colon, breast, and prostate carcinomas showed moderate to high expression of beta 2m. Selective absence of beta 2m expression by certain neuroendocrine tumors appears to be a phenomenon of biological and diagnostic importance.
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PMID:Paucity of beta 2-microglobulin expression on small cell lung cancer, bronchial carcinoids and certain other neuroendocrine tumors. 352 83

The potential concentration of I 131-Meta-iodo-benzylguanidine (MIBG) in pheochromocytoma, and the successful application in diagnosis and therapy of pheochromocytoma, has led to its use in therapy in other tumours derived from the neural crest. In neuroblastoma, the concentration of MIBG is as reliable as it is in pheochromocytoma. 18 patients with a neuroblastoma were treated, leading to two complete remissions, seven partial remissions, two no change, and two progressive disease; one patient was lost for follow-up. Six adults were treated, three with a carcinoid, two malignant pheochromocytoma and one medullary thyroid carcinoma. Although follow-up is still short, preliminary results of therapeutic use of I 131-MIBG indicate that this treatment modality may be effective.
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PMID:Radionuclide therapy of neural crest tumors. 365 6


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