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Query: UMLS:C0027819 (neuroblastoma)
 27,800 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The purpose of the present study was to analyze the clinical pattern of neuroblastoma, the development of therapy, therapeutic results and the effect of neuroblastoma treated in childhood on the patient's later life. A retrospective series of 88 patient under 15 with primary neuroblastoma from years 1951-1978 was analyzed. The material was divided into two groups: patients admitted in 1951-1964 and those admitted in 1965-1978. Thirty three of the children (37.5%) were treated in 1951-1964 and 55 (62.5%) in 1965-1978. No statistical differences were seen between the therapeutic groups regarding age and sex distribution, site of the primary tumor and the histological and stage distribution. The mean age of all the patients on admission was 3.2 years (range 0-14.6 years). The age distribution was as follows: 24 (27.3%) 12 months, 18 (20.5%) 12-24 months and 46 (52.3%) over 24 months. There were 45 (51.1%) boys and 43 (48.9%) girls. All the histological specimens were reexamined. On the basis of histological differentiation, the series contained 67 (76.1%) neuroblastomas and 21 (23.9%) ganglioneuroblastomas. The localization of the primary tumors was the following: neck one (1.1%), mediastinum 17 (19.3%), elsewhere in the thorax 9 (10.2%), abdomen 15 (17.0%), pelvis 6 (8.8%), adrenal glands 31 (35.2%), dumbbell tumors 9 (10.2%). The stage distribution (Evans et al., 1971) was: stage I 6 (6.8%), stage II 20 (22.7%), stage III 36 (40.9%), stage IV 20 (22.7%) and stage IV-S 6 (6.8%). The diagnostic studies performed were chest roentgenography on 97.7%, skull roentgenography on 62.5%, pelvic roentgenography on 18.2%, spinal roentgenography on 23.8%, roentgenography of the long bones on 53.4%, and urography on 59.1% of the total. A bone marrow study was made on 36.4% of the cases. 24-hour urinary excretion of VMA was determined at the beginning of therapy for 78.2% and of HVA for 38.2% of the patients of the latter treatment group. The commonest general symptoms were anemia (34.1% of all patients), vague pyrexia (25.0%), lack of appetite, weight loss (18.2%) and poor general condition (15.9%). General symptoms were commonest in patients in stages IV and III (75.0 and 72.2%) and least frequent in stage I patients (16.7%). The average duration of the general symptoms in the survivors was 1.9 months and in the patients who died 1.7 months.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Neuroblastoma in 88 children. Clinical features, prognostic factors, results and late effects of therapy. 667 Aug 48

A case of esthesioneuroblastoma, the pathological diagnosis of which almost always causes great difficulties, was investigated ultrastructurally, biochemically, and immunohistologically, using antibodies against the five known types of intermediate filaments [keratin, vimentin, desmin, glial fibrillary acidic protein (GFAP) and neurofilaments]. The tumour cells did not react with antibodies against any of the five intermediate filament proteins. Ultrastructural investigations showed dense cored secretory granules in the cytoplasm and cell processes. Thus, immunohistology offers by "exclusion" a differential diagnosis to avoid often misdiagnosed tumours (undifferentiated carcinomas, embryonal rhabdomyosarcomas, and malignant lymphomas), since carcinomas react with antikeratin, embryonal rhabdomyosarcomas with antibodies to desmin and malignant lymphomas show immunofluorescence with antibodies to vimentin. The biological behaviour (age distribution, tendency to metastasize), the normal values of biochemical parameters, homovanillic acid and vanilmandelic acid (HVA, VMA), and the absence of neurofilaments distinguish this type of tumour from the peripheral sympathetic neuroblastoma.
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PMID:Esthesioneuroblastoma: ultrastructural, immunohistological and biochemical investigation of one case. 671 29

This study was undertaken to better define the relationship between catecholamine (CAT) metabolism within the malignant neuroblast and other factors known to influence clinical outcome in neuroblastoma (NB). Several CATs and their metabolites were measured in 32 tumors and 47 urines from 60 newly diagnosed children with neurogenic tumors. Absolute and relative CAT concentrations were correlated with age, clinical stage, histologic differentiation and survival duration. The major findings are: (1) urinary CAT excretion patterns often differ markedly from tumor CAT patterns in the same child, which may be explained by the continuous nature of tumor metabolism, hepatic and renal catabolism, and sampling errors; (2) definite patterns of biochemical maturation are apparent in tumors and urines, but standard light microscopy is insufficient to correlate this with degree of histologic differentiation; (3) more differentiated patterns of CAT metabolism, both in tumor and urine, are significantly associated with age less than 1 year at diagnosis, and with more favorable clinical Stage (I, II, IVS). Tumor NE was increased relative to DM and DA, and urine NMN and VMA were increased relative to DM and HVA, in these patients. (4) this more differentiated CAT pattern in infants and in less advanced stages was reflected in their better survival. Greater maturity of tumor enzyme systems is the probable reason for the better prognosis of NB in infants less than 1 year. Individual enzymes should be measured in fresh tumor tissues, and related to relative and absolute CAT concentrations in tumor and urine, to try to categorize patients on the basis of clearcut biological differences when planning new treatment strategies for NB.
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PMID:Tumor and urine catecholamines (CATs) in neurogenic tumors. Correlations with other prognostic factors and survival. 682 49

The assessment of catecholamines (NE, E), essential metabolites (VMA, HVA) and serotonergic metabolite 5HIIA in urine by HPLC with UV detection is of interest for clinical diagnosis concerning HTA, pheochromocytoma, neuroblastoma, carcinoid syndrome, hypotension, etc. In the same urine sample one can detect (following preliminary steps) by HPLC the above--mentioned biochemical parameters with good reproducibility and sensitivity.
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PMID:An improvement of the endocrine diagnosis by high-performance liquid chromatography. 753 56

The actions of two omega-conopeptides on high-voltage-activated calcium channel currents in differentiated human neuroblastoma IMR32 cells were investigated. Similar to the previously reported action of omega-conopeptide GVIA, omega-conopeptide MVIIA irreversibly blocks IMR32 HVA calcium channel currents at low concentrations. Unlike GVIA action, however, novel omega-conopeptide SNX-260 (iodinated MVIIC) reversibly blocks these currents, also at low concentrations, with an IC50 near 50 nM. Different omega-conopeptides may be potent blockers of HVA calcium channel currents yet act either reversibly or irreversibly in a single cell.
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PMID:Irreversible and reversible blockade of IMR32 calcium channel currents by synthetic MVIIA and iodinated MVIIC omega-conopeptides. 760 42

IgGs from two LEMS patients applied to human neuroblastoma IMR32 cells reduced the density of low- (LVA; T) and high-threshold (HVA; L and N) Ba2+ currents by different percentages: 36% (LVA) and 56% (HVA) for one and 48% and 45% for the other. A pharmacological assay of IgGs action based on the block of L-type channel by nifedipine and on the delayed activation of N-type channel by noradrenaline, indicated a preferential inhibition of the N-type current in IMR32 cells (55% and 47% for the two patients). The L-type current, contributing to approximately one-third of the total, was also depressed by LEMS IgGs but to a minor degree (49% and 30%). Except for an increase of single N-type channel inactivation, LEMS antibodies preserved the elementary properties of single HVA channels, suggesting that the macroscopic current reduction after IgGs treatment is likely due to a decrease in the number of active HVA Ca2+ channels.
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PMID:Inhibition of low- and high-threshold Ca2+ channels of human neuroblastoma IMR32 cells by Lambert-Eaton myasthenic syndrome (LEMS) IgGs. 789 70

We tried to discriminate between cases of spontaneous regression and non-regression during mass screening for neuroblastoma, taking advantage of differences in respect to the urinary homoranillic acid/vanillyl mandelic acid (HVA/VMA) ratio and the original tumor site among true positive, false negative and natural occurrence cases. After classifying them into a total of six groups depending on the two factors, ratio: < 1, 1-2 or > or = 2 and tumor site: adrenal or extra-adrenal in origin, we calculated the mathematical probability of a given true positive case being one of spontaneous regression. A tumor of extra-adrenal origin was likely to regress spontaneously, especially one with an HVA/VMA ratio < 1 or > or = 2 (82.2-100%). A tumor of adrenal origin with an HVA/VMA ratio < 1 seemed unlikely to regress spontaneously (0-4%). The present method, employing simple preoperative information, would be useful in future for the selection of true positive cases which should be observed without treatment.
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PMID:A trial to discriminate spontaneous regression from non-regression cases during mass screening for neuroblastoma. 796 3

1) By March 1992, 166,959 infants were screened in Sapporo City, and 35 patients were found to have this tumor. Incidence of this tumor in our screening was one among 5,059 babies examined. 2) Eleven infants among the group with negative screening at 6 months developed neuroblastoma later. The patients developed tumors one to several years after mass screening, and 2/3 of these cases showed high urinary HVA compared to VMA. 3) We postulate that some of the adrenal steroids, possibly cortisol, may play a role for the malignant progression of this tumor.
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PMID:Effects of mass screening for neuroblastoma and the presumptive natural history of this tumor. 797 35

Cervical neuroblastoma is relatively rare and shows very good prognosis. We reported a case of surgically resected cervico-mediastinal neuroblastoma. A 7-month-old boy was pointed out to have Horner syndrome (right ptosis and anhidrosis) and an abnormal shadow at right cervico-mediastinum on chest X-ray film during his stay in another hospital because of acute bronchitis. On examination after admission in our hospital, 3 x 4 cm size mass was palpated at the right neck and urine VMA and HVA level were elevated. The close examination demonstrated no sign of tumor in any other organ and tissue, which suggested that the tumor was cervical origin. 9 x 5, 5 x 3 cm tumor was almost removed and patient received chemotherapy. He is now free from disease at 1 year after operation.
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PMID:[Experience with a operated cervico-mediastinal neuroblastoma in infant]. 830 10

It was found a solid tumour of 24 x 28 x 31 mm size above the right kidney with ultrasound screening in a three days old newborn. After three days appeared cystic areas inside the tumour, so it was thought to adrenal haemorrhage. The tumour didn't decrease during two months and metastases developed in the liver. The urinary VMA level was normal, the HVA elevated. The MIBG scintigraphy was first negative, after a month pathognostic. The primer tumour and metastases disappeared after cyclophosphamid and Adriablastin treatment. The adrenal neuroblastoma can imitate the adrenal apoplexy, so it's important to control all neonatal adrenal haemorrhage until their disappearance.
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PMID:[Neonatal neuroblastoma detected by ultrasonic screening]. 823 51


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