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)

Synaptophysin, an Mr 38,000 integral membrane glycoprotein of neurotransmitter vesicles, has been identified in diverse primary neuroendocrine (NE) tumors of both neural and epithelial origin (Wiedenmann and co-workers, Proc Natl Acad Sci USA 1986; 83: 3500-3504). In the present study, metastases of several types of NE tumors, including medullary thyroid carcinoma, gastrinoma, insulinoma, small (oat) cell carcinoma of the lung, gastrointestinal carcinoid, and neuroblastoma, were examined for the presence of synaptophysin by immunocytochemistry, with the use of tissue sections as well as centrifuged cell suspensions and by immunoblotting of tumor proteins. The results show that expression of synaptophysin can be maintained during formation of metastases. Therefore, the authors propose that synaptophysin antibodies be used for the positive identification of metastatic NE tumors, notably in differential diagnosis. The possible implications of these findings for tumor diagnosis are discussed.
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PMID:Synaptophysin identified in metastases of neuroendocrine tumors by immunocytochemistry and immunoblotting. 311 96

A patient in whom metastatic medullary thyroid cancer was diagnosed underwent a scintigraphic examination using [131I]MIBG. Multiple hot lesions and diffuse hepatic uptake were noted corresponding to bone and liver metastases. Iodine-131 MIBG may prove to be useful for scintigraphic localization and for the treatment of medullary thyroid cancer as in pheochromocytoma and neuroblastoma.
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PMID:Metastatic medullary thyroid cancer: localization with iodine-131 metaiodobenzylguanidine. 315 26

Neuron-specific enolase (NSE) is an enzyme detectable in nervous and neuroendocrine tissue. Increased serum levels of NSE are found in small cell lung cancer and in patients with neuroblastoma, in whom NSE is used as a serum tumor marker. We have investigated 32 patients with histologically proven medullary thyroid carcinoma, a tumor of neuroendocrine origin, in which the classical tumor marker calcitonin (CT) was pathologically elevated. Positive immunocytochemistry for NSE and CT in C-cells was obtained in all cases. Increased serum NSE levels were found in only 5 of 32 patients, there was no correlation between NSE and CT concentrations. We also compared NSE and CT serum levels during long-term follow-up and again found no correlation between NSE and CT. After i.v. stimulation tests with pentagastrin and calcium, no correlation was found between NSE and CT serum levels. We conclude, therefore, that in medullary thyroid carcinoma NSE is useful for immunocytochemistry but not a reliable serum tumor marker.
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PMID:Neuron-specific enolase in medullary thyroid carcinoma: immunohistochemical demonstration, but no significance as serum tumor marker. 331 43

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

The syndrome of opsoclonus-myoclonus (OM) is an infrequent but well-known "remote effect" of neuroblastoma in children. The OM syndrome is even less frequent in adults. A few cases of adult paraneoplastic OM have been described in association with several systemic neoplasms. We report the unique case of a 29-year-old man with metastatic medullary thyroid carcinoma in whom OM developed as part of a generalized transient encephalopathy. We outline the postulated anatomic lesions and pathophysiologic mechanisms underlying the OM syndrome, as well as examine the possible connections between the neuroendocrine derivation of medullary thyroid carcinoma and the neurotoxic and/or autoimmune theories of the causation of the OM syndrome in patients with systemic neoplasms.
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PMID:Paraneoplastic opsoclonus-myoclonus. Association with medullary thyroid carcinoma and review of the literature. 395 25

The details of a radioimmunoassay capable of measuring as 5 pg of prostaglandin A, E, and F (PGA, PGE, and PGF) in human and rat plasma are described. Plasma samples are extracted (with 4000 cpm [(3)H] PGE(1) added for calculation of recovery) with an organic solvent system at an apparent pH of 5.8 and then chromatographed on silicic acid columns with increasing concentrations of methanol to separate PGA, PGE, and PGF. Each chromatographed sample is measured by radioimmunoassay, using the homologous antibody and tritiated marker. 40 normal individuals had mean plasma concentrations of PGA, PGE, and PGF of 1062+/-107 pg/ml, 385+/-30 pg/ml, and 141+/-15 pg/ml, respectively. Elevated PGE levels were measured in the plasma of patients with medullary carcinoma of the thyroid, carcinoid, and neuroblastoma. Treatment of rats with indomethacin decreased serum PGE levels by 67%. The radioimmunoassay appears to be of considerable experimental as well as clinical interest.
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PMID:Radioimmunoassay measurement of prostaglandins E, A, and F in human plasma. 468 79

The increased understanding of the neuroendocrine tumors at a cellular and molecular level has led to the development of new radiopharmaceuticals for imaging. Two of the imaging agents include 131I metaiodobenzylguanidine (131I-MIBG) and 111In-DTPA-D-Phe1-octreotide (111In-pentetreotide) each having specific localization in certain neuroendocrine tumors. The selective uptake of these radiopharmaceuticals by the tumor cells has generated interest in potential use for targeted radiotherapy for neuroendocrine tumors. 131I-MIBG has been used to treat patients with pheochromocytoma, neuroblastoma, carcinoid tumors, medullary thyroid carcinoma, and paragangliomas. The tumor responses have been variable with the most encouraging results being in patients with pheochromocytoma. The dose-limiting toxicity has been thrombocytopenia or granulocytopenia. 111In-pentetreotide has been used as therapy in only a few patients and has resulted in objective evidence of tumor responses. A therapeutic agent using a somatostatin analogue will most likely require radiolabeling with a beta- or possibly an alpha-emitting radionuclide to achieve significant and durable tumor responses.
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PMID:Therapy of neuroendocrine tumors with radiolabeled MIBG and somatostatin analogues. 757 46

From January 1993 to January 1994, scintigraphy with 123I-MIBG and/or 131I-MIBG were performed in 22 patients and their scintigraphic usefulness was evaluated. Iodine-123 MIBG and 131I-MIBG scintigrams were obtained 24 hours after injection of 222 MBq of 123I-MIBG and 48 hours after injection of 20 MBq of 131I-MIBG, respectively. In two patients with pheochromocytoma, the 123I-MIBG and 131I-MIBG scans were performed and both images were compared. In a patient with single intraadrenal pheochromocytoma, the lesion not detected with 131I-MIBG was clearly visualized with 123I-MIBG. In the other patient with multiple metastatic pheochromocytoma, much more lesions were distinctly demonstrated on the 123I-MIBG images than on the 131I-MIBG images. All of the lesions were detected with 123I-MIBG in a patient with pheochromocytoma, 3 patients with neuroblastoma and a patient with medullary thyroid cancer. Most of the normal adrenal glands (86%) were visualized on the 123I-MIBG scintigrams, in 7 patients without neural crest tumor and adrenal diseases, while 131I-MIBG scintigraphy failed to visualize normal adrenal glands in 2 hypertensive patients. The main reason for the superiority of 123I-MIBG to 131I-MIBG is considered to be as follows: 1) higher specific activity of 123I-MIBG. 2) the larger amount of 123I-MIBG used. 3) gamma ray energy of 123I is ideal for gamma camera. In conclusion, 123I-MIBG appears to be a more suitable imaging agent than 131I-MIBG in depicting neural crest tumors.
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PMID:[Detection of neural crest tumors by 123I-MIBG scintigraphy]. 783 4


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