Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0027819 (neuroblastoma)
 27,800 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

cis-Dichlorodiammineplatinum(II) (cis-platinum) was evaluated in three separate studies at Roswell Park Memorial Institute in children and adolescents with cancer. In the first study, 16 patients with a variety of solid tumors were treated. Objective responses were seen in patients with neuroblastoma, osteogenic sarcoma, seminoma, and medullary carcinoma of the thyroid. In the second study, five of eight patients with far-advanced osteogenic sarcoma showed objective responses to cis-platinum. In the third study, cis-platinum and Adriamycin were employed as primary adjuvant chemotherapy along with surgery in osteogenic sarcoma. Nine of ten patients have remained disease-free from 8 to 31 months (mean, 19 months). cis-Platinum is an active agent in pediatric tumors.
 ...
PMID:cis-Dichlorodiammineplatinum(II) in childhood cancer. 29 83

Abnormalities of neural crest development may be manifested in a wide variety of ways ranging from von Recklinghausen's disease to Sipple's syndrome -- pheochromocytoma and medullary thyroid carcinoma. We report here yet another manifestation -- coexistent multiple neuroblastomas and Hirschsprung's disease in a newborn. In a review of the English literature we have found the combination of neuroblastoma and Hirschsprung's disease recorded only once previously [2]. However, the relationship between the two diseases was not discussed. The two diseases may be at the extremes of a spectrum of neural crest development pathology, i.e., Hirschsprung's representing a deficiency and neuroblastoma representing malignant proliferation of cells of neural crest origin.
 ...
PMID:Coexistent neuroblastoma and Hirschsprung's disease--another manifestation of the neurocristopathy? 47 59

cis-Dichlorodiammineplatinum(II) (DDP) was studied in 16 children with far-advanced malignancies. Three dosage schedules were tried: regimen A, 20 mg/m2/day x 5 days for 3-4 weeks (11 patients); regimen B, 50 mg/m2 once a week (four patients); and regimen C, 60 mg/m2/day x 2 days every 3-4 weeks (one patient). Four of 16 patients (25%) showed partial response, including one with osteogenic sarcoma, one with neuroblastoma, one with seminoma, and one with medullary carcinoma of the thyroid. Two patients showed clinical improvement. The major toxic manifestations included nausea and vomiting (16 of 16), renal toxicity (three of 16), transient pancytopenia (six of 12), and hearing loss (two of 16). It is apparent that DDP has activity in pediatric tumors; however, a more precise response rate must be delineated in a larger series of patients.
 ...
PMID:Clinical response and toxicity with cis-dichlorodiammineplatinum(II) in children. 89 Jun 92

A phase III clinical study of 131I-metaiodobenzylguanidine (131I-MIBG) was performed in 66 patients with tumors of sympathetic and adrenomedullary origin, including 32 patients with suspected pheochromocytoma, 25 with suspected neuroblastoma, 7 pre- or postoperative medullary carcinoma of the thyroid and each with carcinoid and suspected Sipple's syndrome. A total of 150 sites which were confirmed for presence (72 sites) or absence (78 sites) of tumors were examined on 131I-MIBG scintigrams. True positive ratio of the scintigraphy was 84.7% (61/72) and true negative ratio was 94.9% (74/78). Positive scintigraphy was obtained in 86.5% (32/37) of pheochromocytoma, 78.6% (22/28) of neuroblastoma and 100% (6/6) of medullary carcinoma of the thyroid. Accumulation of 131I-MIBG was seen in 16.8% of normal adrenal glands. Neither adverse reactions nor abnormal laboratory findings were noted in relation to 131I-MIBG injections. Our study indicates that 131I-MIBG is a safe and clinically useful radiotracers for the visualization and localization of tumors of sympathetic and adrenomedullary origin.
 ...
PMID:[The assessment of clinical usefulness of 131I-MIBG scintigraphy for localization of tumors of sympathetic and adrenomedullary origin--a report of multicenter phase III clinical trials]. 136 May 49

We have presented a case of sporadic medullary carcinoma of the thyroid with documentation of localization of tracer 131I-MIBG within the primary neoplasm. A review of the nuclear medicine literature of localization techniques for MCT demonstrates that 131I-MIBG, while an excellent choice for diagnosis of pheochromocytoma and neuroblastoma, produces low yield and unpredictable concentration in other neural crest apudomas, including MCT. A low incidence of true-positive results with 131I-MIBG uptake and a high incidence of false-negative results make this radiopharmaceutical a suboptimal choice for diagnostic studies, but a potentially promising one as a therapeutic agent.
 ...
PMID:Iodine 131 metaiodobenzylguanidine scintigraphy of medullary carcinoma of the thyroid. 185 77

Sixteen tumor markers are reviewed, and measured to the ideal: produced by the tumor cell alone absent in health and in benign disease present in all patients with a given malignancy level in the blood representative of tumor mass detectable in occult disease. The only marker that approaches the ideal is human chorionic gonadotropin (HCG) in gestational trophoblastic tumors. In this malignancy, the HCG level suggests the diagnosis and stage, confirms response to therapy, and predicts relapse. The three most widely used and intensely studied tumor markers are carcinoembryonic antigen (CEA), alphafetoprotein (AFP), and HCG. CEA cannot be used in screening for cancer, but in carcinoma of the colon its elevation preoperatively increases the likelihood of advanced disease and postoperative recurrence. Postoperatively, elevated titers are often but not invariably associated with recurrent disease. AFP and HCG are useful in the management of nonseminomatous germ cell testicular tumors. Like CEA, they cannot be used for screening. They are more likely to be increased with advancing stage, and after therapy rising levels almost always mean recurrent disease. Some markers are valuable in specific circumstances, such as calcitonin in screening for familial medullary carcinoma of the thyroid. In multiple myeloma, immunoglobulins are useful in determining the tumor mass and response to therapy. In neuroblastoma, catecholamine metabolites are useful primarily in making the diagnosis. In some malignancies, the absence of effective therapy lowers the value of the marker, as for AFP in hepatoma. The remaining markers are too unreliable or too little studied to be useful in the management of an individual patient with cancer. The purpose of this paper is to provide the clinician with an understanding of the limitations of the present tumor markers that will lead to wiser use of the tests, and to provide standards to which future tumor markers should be measured.
 ...
PMID:Tumor markers: value and limitations in the management of cancer patients. 241 41

Partial monosomy of the short arm of chromosome 1 is the most consistent cytogenetic abnormality found in human neuroblastomas, but its overall frequency and significance are unclear. Using a panel of chromosome-1-specific DNA probes that identify restriction fragment length polymorphisms, we demonstrate that 13 of 47 human neuroblastomas (28%) have somatic loss of heterozygosity (LOH) at one or more loci on the distal short arm of chromosome 1. the chromosomal region that shows LOH most consistently is between 1p36.1 and 1p36.3; loss of a gene or genes in this region may be critical for the development or progression of neuroblastomas. The region of LOH in human neuroblastoma may resemble that described for pheochromocytoma, medullary thyroid carcinoma, and melanoma, which are also tumors of neural-crest origin. Although LOH for distal chromosome 1p can occur in early stages of neuroblastoma, the loss usually occurs in tumors of advanced clinical stages. LOH for the short arm of chromosome 1 correlates significantly with N-myc amplification, suggesting that these two genetic events are related. Indeed, these two lesions appear to characterize a genetically distinct subset of particularly aggressive neuroblastomas.
 ...
PMID:Loss of heterozygosity for the short arm of chromosome 1 in human neuroblastomas: correlation with N-myc amplification. 256 96

We have found that neuroendocrine tumors (including neuroblastoma, ganglioneuroma, gut carcinoid, pheochromocytoma, medullary thyroid carcinoma, insulinoma, glucagonoma, prolactinoma, carotid body tumor, and small cell lung carcinoma) produce considerable amounts (about 1000-80,000 ng/g tissue) of the alpha subunit of guanine nucleotide-binding protein, GO (GO alpha), whereas nonneuroendocrine tumors contain less than 300 ng of GO alpha/g tissue. GO alpha in the neuroendocrine tumors was present both in the soluble fraction, and cholate-extractable membrane-bound fraction of tissues. Immunoblots of membrane fractions of neuroblastoma and carcinoid tissues confirmed that the immunoreactive substance in the tumor tissues was GO alpha. Immunohistochemically, GO alpha was localized consistently in the cell membrane and occasionally in the cytoplasm of neuroendocrine tumors. GO alpha was also detected in sera of 73% patients with neuroblastoma at diagnosis, whereas serum GO alpha concentrations in control children, or patients with nonneuroendocrine tumors were lower than the detection limit of the immunoassay method employed. Serum GO alpha concentrations in patients with neuroblastoma changed with the clinical course; they fell in patients responding to treatment and increased in patients who relapsed. Since GO alpha, a specific protein in the neural and neuroendocrine cells, was found to be produced in considerable amounts by all types of neuroendocrine tumors but not in nonneuroendocrine tumors, GO alpha might be a useful biomarker for neuroendocrine tumors.
 ...
PMID:Production of the alpha subunit of guanine nucleotide-binding protein GO by neuroendocrine tumors. 282 34

While 131I-meta-iodobenzyl guanidine (131I-MIBG) scanning has made possible the scintigraphic visualization of pheochromocytoma and neuroblastoma, an accumulation of this agent has recently been reported in medullary thyroid cancer. We present the case of a patient with Sipple's syndrome (multiple endocrine neoplasia type IIa), in whom we were able to identify distant metastases and local invasion of medullary thyroid cancer as well as primary thyroid tumour and right adrenal pheochromocytoma, using 131I-MIBG scans. This case highlights the usefulness of 131I-MIBG in the detection of metastatic medullary thyroid cancer and suggests that this agent may also be of therapeutic use in the treatment of tumours.
 ...
PMID:Detection of metastatic medullary thyroid cancer with 131I-MIBG scans in Sipple's syndrome. 287 28

131I-meta-iodobenzylguanidine (131I-MIBG) was also taken up by medullary thyroid carcinoma (MTC) as well as by pheochromocytoma in two patients with Sipple's syndrome. However, the mechanism of 131I-MIBG uptake by MTC has not been clarified yet. We measured tissue catecholamine levels in three MTC, since MTC can produce several active substances. Catecholamines were detected in various amounts in all MTC, but not in normal thyroid tissues. These findings suggest that MTC can produce catecholamines and therefore, 131I-MIBG is taken up and stored in catecholamine vesicles of MTC, like pheochromocytoma and neuroblastoma. We conclude that 131I-MIBG may be applied not only to diagnosis but also for the treatment of patients with MTC.
 ...
PMID:Imaging and uptake mechanism of 131I-meta-iodobenzylguanidine in medullary thyroid carcinoma. 287 69


1 2 3 4 5 6 Next >>