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)

We performed chromosome analysis of 15 neuroblastomas found by mass screening using a vanillymandelic acid spot test. We found near triploid chromosome abnormalities, ranging from 60 to 77 chromosomes, in the tumor cells from 14 patients, and hyperdiploidy with the mode of 50 in cells from one. A structural abnormality was observed in only one patient. We did not find a marker chromosome 1, homogeneously staining region, or double minutes, which have been previously reported in advanced neuroblastomas or in cell lines. All of our patients were completely free of disease 4 to 32 months after diagnosis. We consider that patients with hyperdiploidy or near triploidy are different from those with marker chromosome 1, homogeneously staining region, or double minutes and may constitute a subgroup with a good prognosis in childhood neuroblastoma.
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PMID:Chromosome findings and prognosis in 15 patients with neuroblastoma found by VMA mass screening. 335 79

The intratumorous distribution of catecholaminergic tumour cells and S100 protein-positive elements of 8 neuroblastomas, 8 ganglioneuroblastomas and 2 ganglioneuromas were studied using catecholamine fluorescence and immunohistochemical staining, respectively. The elements of catecholamine fluorescence were observed in all tumour specimens, even in urinary VMA-negative cases, but the patterns of distribution were not uniform. Catecholamine fluorescence was observed in both tumour cell bodies and neurofibres, and the appearance of the latter but not that of the former correlated with the histological grade of differentiation, thereby suggesting the occurrence of catecholaminergic differentiation within the tumour. S100 protein-positive elements also correlated with the histological differentiation, and were distributed mostly in the area where the catecholamine-containing neurofibres were located. The possible correlation between catecholaminergic differentiation and the appearance of S100 protein-positive elements in neuroblastoma requires close attention.
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PMID:Catecholaminergic differentiation associated with S100 protein-positive elements in human neuroblastoma. 353 92

A newborn with symptoms of massive adrenal hemorrhage is presented. The cause was a unilateral neuroblastoma of the adrenal gland, bringing about severe anemia and hemorrhagic shock. The suspicion of a tumor of the adrenal gland was confirmed by infusion urography, ultrasound examination of the kidney, and the increased amount of vanillymandelic acid in the urine. Nephrectomy was performed on the fourth day of the newborn's life. The tumour was classified in the IV-S stage after Evans et al. Following the operation, neither cytostatics nor radiation was applied. The child is developing normally and the amount of vanillymandelic acid in the urine is within normal limits. The child is now in the 16th month of life.
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PMID:[Massive adrenal hemorrhage in a neonate due to neuroblastoma]. 356 Oct 28

There is still controversy regarding the relative merits of catecholamine metabolite estimations on 24 h versus untimed urine collections. The former has the advantage of taking into account diurnal variation in the rate of metabolite excretion but has the disadvantages of delaying results and of being affected by errors in collection. In this study percentile values were established for a reference population of 181 children for urinary 4-hydroxy-3-methoxyphenylacetic acid (HVA)/creatinine and 163 children for 4-hydroxy-3-methoxymandelic acid (VMA)/creatinine, using untimed urine collections. Results of similar determinations performed as part of the diagnostic work up of 23 consecutive children subsequently proven to have neural crest tumours showed that all patients had the value of at least one metabolite concentration at or above the highest reference value. In neuroblastoma all patients' VMA/creatinine exceeded the highest reference value and in neural crest tumours overall, this ratio was greater than the highest reference value in 96% of patients. These results are as good as, or better than, previously published results and demonstrate the practical value of using catecholamine metabolite determinations expressed as 'creatinine equivalents' on untimed urine specimens in the diagnosis of neuroblastoma and related tumours in children.
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PMID:The value of catecholamine metabolite determination on untimed urine collections in the diagnosis of neural crest tumours in children. 356 81

The importance of determination of serum neuron-specific enolase (NSE) in patients with neuroblastoma has been emphasized by several authors. However, the specificity and sensitivity of NSE have not yet been well studied in tumors of infancy and childhood, nor is the role of serial determination of NSE in monitoring these patients fully understood. Concentrations of serum NSE were determined by a newly developed radioimmunoassay technique in 241 samples from 111 patients. NSE was also assayed in sera of nude mice bearing human pediatric tumors (16 samples), as well as in 30 tumor specimens. Eighty-two serum samples from 19 patients with neuroblastoma all showed NSE values (mean 120.2 ng/mL, range 16.2 to 722.0 ng/mL) elevated beyond the upper border of the normal range (14.6 ng/mL), even though four of the 19 patients had normal urinary excretion of 3-methoxy-4-hydroxymandelic acid (VMA) and 3-methoxy-4-hydroxy-phenylacetic acid (HVA). Twelve of these patients were monitored with serial NSE determinations, and their serum NSE were found to correlate well with the tumor burden, but were transiently modified by chemotherapeutically induced cell death. All 68 samples from nine patients, free of neuroblastoma at assessment, showed NSE values within the normal range. Thirteen of 25 patients with tumors other than neuroblastoma, however, showed serum NSE values mildly elevated beyond the upper border of the normal range (mean of the 25 patients 36.7 ng/mL, range 5.0 to 234.0 ng/mL). Results from our nude mouse study and from NSE analysis of the tumor extracts paralleled the clinical results.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Serial determination of serum neuron-specific enolase in patients with neuroblastoma and other pediatric tumors. 358 64

The clinical manifestations of 253 neuroblastoma cases in childhood, treated in Denmark from 1943 to 1980, were reviewed. Most striking was the vagueness of symptoms in the majority of patients, only a few of whom exhibited the symptoms strongly suggestive of a neuroblastoma (i.e. the Horner syndrome, the watery diarrhoea syndrome, the dancing eye syndrome). The vagueness of the symptoms might have led to fatal procrastination of the diagnosis. The diagnostic delay has, however, no independent prognostic significance for survival in our patients (p = 0.09). The maximal tumour spread was recorded for all 253 patients, and the distribution of metastases was in accordance with the "soil-seed" hypothesis. The tumour spreads with equal frequency by local growth, by lymphatic vessels to distant lymph nodes, and by blood to bone. Only in widely disseminated tumours are metastases to the lungs, the meninges, the brain, and the reproductive organs seen to occur. Eighty-five percent of the patients, for whom data were available, excreted VMA above the normal value for their age, and 43% excreted Norepinephrine + Epinephrine (N + E) above normal levels. The excretion of both VMA and N + E was significantly correlated to stage, and thus to prognosis. Neither the level of VMA excretion nor the level of N + E had any bearing on the survival when age and stage were adjusted for. Serial VMA and N + E determinations show that patients with normal values for these parameters had significantly better prognosis than patients with elevated values during the first, second, third and fourth trimesters after the initiation of treatment. Increasing values in the individual patient were associated with a poor prognosis. We found no correlation between the initial leucocyte count and survival when age and stage were adjusted for.
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PMID:Signs, symptoms, metastatic spread and metabolic behavior of neuroblastomas treated in Denmark during the period 1943-1980. 363 7

This is a review of 80 patients with neuroblastoma managed at the American University of Beirut Medical Center between 1963 and 1983. Three patients had ganglioneuroblastoma of whom one showed histologic evidence of maturation into a ganglioneuroma. Four patients were less than 1 month of age and 33 were less than 2 years of age. The site of origin was intra-abdominal in 56 patients of whom 34 were intra-adrenal. Intraspinal involvement was noted in 12 patients, of whom one was a newborn. Treatment and adequate follow-up were possible in 63 patients. Total excision of the tumor was performed in 17 patients, and partial excision in 14. The 2-year and 5-year survival rates were 36% and 25%, respectively. Age, site of the tumor, and degree of cellular differentiation were the only independent variables affecting survival. Eleven of 14 patients younger than 1 year were alive 5 or more years after diagnosis. Cervical, thoracic, and pelvic tumors had a better prognosis than abdominal tumors. Other factors affecting survival were the stage and the mode of therapy. Infants with stage IV-S congenital neuroblastoma had a very poor prognosis. The initial urinary VMA level as well as the presence or absence of calcifications within the tumor had no bearing on prognosis.
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PMID:Neuroblastoma: report on a 21-year experience. 371 88

Neuron-specific enolase (NSE) in sera of 3 patients with neuroblastoma (Stage IV) were measured by radioimmunoassay, as compared with urinary catecholamine metabolites (vanillyl-mandelic acid (VMA) and homovanillic acid (HVA] during the course of chemotherapy, radiation, and second look operation. In Case 1 (Stage IV B) and Case 3 (Stage IV A), NSE-level on admission was found to be elevated to 51.0 ng/ml and 25.5 ng/ml, respectively. VMA and HVA were also elevated. In Case 2 (Stage IV A), NSE on admission was elevated to 128.0 ng/ml., HVA was high, but VMA was within normal range. From 1 to 3 weeks after chemotherapy and radiation, high levels of urinary VMA and/or HVA in patients promptly decreased within normal range. The size of primary tumor masses either showed no marked change or slightly decreased by radiological examinations. After intensive chemotherapy, high levels of serum NSE decreased within normal range. At that time, second look operations were carried out. The size of primary tumors was reduced (3.6 X 2.7 X 2.1 cm in average) and almost all masses had scarred over. These data suggest that serum NSE levels correlate very well with residual tumor burdens.
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PMID:Serum neuron-specific enolase as a marker useful for monitoring the effectiveness of therapy in patients with neuroblastoma--as compared with urinary catecholamine metabolites. 373 14

In Nagoya City, Japan, VMA mass screening for detection of neuroblastoma has been performed since 1977. Filter paper strips wetted with urine are mailed to a central laboratory. Positive tests are followed up by more definitive testing. Of the 20,053 urine samples of 6-month-old infants tested, January 1977 to March 1983, five asymptomatic infants with neuroblastoma have been discovered. All have been treated and are alive as of January 1984.
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PMID:VMA mass screening program of neuroblastoma for infants in Nagoya City, Japan. 396 25

We describe a method for determining the "total" excretion of acidic metabolites of catecholamines by measuring 3,4-dihydroxyphenylacetic acid (DOPAC) formed by hydriodic acid hydrolysis of 4-hydroxy-3-methoxyphenylacetic acid (HVA), 4-hydroxy-3-methoxymandelic acid (VMA), and their conjugates. The DOPAC in the diluted hydrolysate is measured directly by liquid chromatography with electrochemical detection. Normal values, expressed in relation to excretion of creatinine, vary as a function of age. For healthy subjects, the mean DOPAC value after hydrolysis was 1.15 times that for unconjugated HVA, VMA, and DOPAC combined. Preliminary results for patients with neuroblastoma and melanoma indicate the potential usefulness of the method for diagnosis and prognosis of patients with neural crest tumors that produce dopa or catecholamines.
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PMID:"Total" acidic metabolites of catecholamines in urine as determined by hydrolysis with hydriodic acid and liquid chromatography: application to patients with neuroblastoma and melanoma. 400 89


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