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)

Two neurotransmitter-synthesizing enzymes, tyrosine hydroxylase (TH) and choline acetyltransferase (CAT), were assayed in neuroblastoma tissues from 24 children, in human neuroblastoma tissues serially transplanted in nude mice, and in human neuroblastoma cells in culture. Among tissues from 24 children, five showed an adrenergic pattern with significant TH activity alone, seven showed a cholinergic pattern with significant CAT activity alone, and the remaining 12 specimens showed a "both-active" pattern with both TH and CAT activity. Enzymatic activities were maintained through many serial passages in vitro and in nude mice. All four specimens from children under one year of age exhibited the adrenergic pattern. In general, enzymatic activity was not correlated with degree of differentiation histologically. Among four cases of paravertebral dumb-bell type in this series, two were cholinergic, one was adrenergic, and the last was both-active. These results suggest that dumb-bell type tumors may arise from either sympathetic ganglia or dorsal root ganglia. This study supports the concept that neuroblastomas are a composite of adrenergic and cholinergic cells. Significant changes in the relative proportions of these two cell types were observed with time, and after extensive therapy. Different metastatic sites often exhibited important differences in enzymatic activity. These results help to account for clinical discrepancies between urinary VMA levels and tumor growth. Assays for TH and CAT can be useful for confirming a diagnosis of neuroblastoma, and have important potential for helping to clarify the natural history of neuroblastoma.
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PMID:Tyrosine hydroxylase and choline acetyltransferase activity in human neuroblastoma. Correlations with clinical features. 613 77

Forty-five cases of neuroblastoma were reviewed to assess the value of current diagnostic methods. Urinary catecholamine and 3-methoxy-4-hydroxymandelic acid levels were elevated in only 48% and 60% of cases, respectively. All abdominal or pelvic tumour masses were detected by intravenous urography, ultrasound or computed tomography (CT): CT was the best single investigation but the two less expensive techniques detected most of the tumours. Trephine biopsy was more successful than aspiration in detecting bone marrow metastases. Liver scintigraphy was positive in six of seven cases with hepatic secondaries.
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PMID:The diagnosis and staging of neuroblastoma. 661 83

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

Since 1974, we have been mass screening for neuroblastoma in 6-month-old infants using a VMA (vanilmandelic acid) spot test in Kyoto City. Fifteen (48.4%) of the 31 childhood neuroblastoma cases registered in this city during the recent 8-year-period, 1974-1982, were under 1 year of age. The annual number of cases per year was 3.9 cases under 15 years with 1.9 cases under 1 year of age. As the number of children under 15 years of age in this city was 292,000 according to the National Census of the Human Population in 1975, the annual incidence of this tumor in childhood was 3.9/292,000 (13.3/million). And as the number of live births was 161,153, the annual detection rate for this tumor in infancy increased to 15/161,153 (93/million) from 1974 to 1982. We found that many neuroblastomas could be detected in infancy by a VMA spot test and/or a careful physical examination.
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PMID:Incidence of neuroblastoma in infancy in Japan. 670 May 44

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

Since July 1973, the authors began developing a mass screening system using a VMA (vanilmandelic acid) spot test on 6-to-7 month-old infants for early detection of neuroblastoma in Kyoto city, Japan. Using this method, six infants with this tumor were discovered; five of the six infants were cured, and one is under treatment. These patients showed a favorable prognosis on early diagnosis. In this article, 57 neuroblastoma patients from the Department of Pediatrics, Kyoto Prefectural University of Medicine, treated during the last 20 years, from July 1962 to June 1982, are evaluated. Since the mass screening program has run smoothly since July 1974, clinical findings are compared between 35 neuroblastoma cases before mass screening from the 12-year period from July 1962 to June 1974 and 22 cases after mass screening, during the 8-year period from July 1974 to June 1982. Before mass screening, only 20% (7/35) of the patients were discovered with neuroblastoma younger than 12 months of age and 68.6% were older than 2 years of age. After mass screening, 54.6% (12/22) of the patients were younger than 12 months of age and only 31.8% (7/22) were older than 2 years of age. Before mass screening, 17.1% (6/35) survived with five of the six surviving patients being younger than 12 months of age at the time of diagnosis; 72.7% (16/22) of the patients detected after mass screening are living now. Eleven of the 16 patients have already been cured, and the remaining 5 patients are presently undergoing treatment. A marked improvement of their prognoses is dependent on the early detection of this tumor by mass screening. To date, using the VMA spot test for early detection in infancy is convenient and effective for improvement of its prognosis.
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PMID:Neuroblastoma. Mass screening for early detection and its prognosis. 672 33

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

Two cases of infantile myoclonic encephalopathy with opsoclonus and neuroblastoma are reported. One of them was observed in a 16-month-old boy and the remaining one in a 13-month-old girl. In both cases the tumour was a stade III abdominal neuroblastoma. Urinary catecolamine excretion was increased in the girl and that of VMA in both patients. Treatment of the tumour with surgery, radiotherapy and chemotherapy resulted in a immediate disappearance of the neurologic picture in the girl. The same measures were equally successful in the boy implemented with a course of ACTH. Both children are free of tumour three years later, and they do not bear any psychomotor sequelae. The main features of this rare association are briefly commented.
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PMID:[Infantile myoclonic encephalopathy with opsoclonus and neuroblastoma. Apropos of 2 cases]. 688 34

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

1. Tyrosine and tryptophan, as well as 26 metabolites of these amino acids, were analyzed simultaneously in urine specimens from patients with neuroblastoma and control infants by a three-dimensional HPLC system to develop an early diagnosis. 2. Levels of detected compounds in urine from patients with neuroblastoma were generally higher in the case of catecholamines and lower in the case of indolalkylamines than those in controls. 3. The pathways of Dopamine-3,4-Dihydroxyphenylacetic acid-Vanillylmandelic acid, Dopamine-3-Methoxy-4-hydroxyphenylethylene glycol-Vanillylmandelic acid and Tyrosine-4-Hydroxyphenylacetic acid-4 were, in particular, found to be active in patients with neuroblastoma.
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PMID:Simultaneous analyses of monoamines and their metabolites in urine specimens of patients with neuroblastoma. 790 Sep 60


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