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)

Previous studies indicating the importance of catecholamine metabolism in neuroblastoma were briefly reviewed. Metabolic pathways were presented showing how the major urinary metabolites 3-methoxy-4-hydroxymandelic acid (VMA) and 3-methoxy-4-hydroxy-phenylacetic acid (HVA) are formed from norepinephrine and from dopamine plus 3,4-dihydroxyphenylalanine (DOPA), respectively. For 289 neuroblastoma patients at the time of diagnosis, the urinary excretion of VMA was significantly elevated in 75%, and HVA was elevated in 80%. Periodic assay of these metabolites during the course of the disease revealed that the excretion trends were of prognostic value with 80-90% reliability. By contrast, when the excretion in only the initial urine specimens was considered, the survival rate was the same for patients with normal, and with significantly elevated, excretion. Review of the results of tracer studies aimed at elucidating the in vivo metabolic origins of the urinary metabolites suggested that a) in neuroblastoma, the catecholamines were largely inactivated by intracellular metabolism in the tumor cells; b) there was excess production and excretion of the norepinephrine precursors, DOPA and dopamine; and c) in the tumors of most neuroblastoma patients, the initial enzyme in catecholamine synthesis, tyrosine hydroxylase, had an activity comparable with that in normal adrenal glands. The importance of the metabolism of catecholamines in patients with neuroblastoma was stressed: a) The excretion of elevated levels of urinary catecholamine metabolites were useful in diagnosis and in following the course of the disease, and b) study of the catecholamine metabolism in these patients permitted examination of possible relationships between the activity of the enzymes involved in catecholamine synthesis and the malignancy of this tumor.
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PMID:Catecholamine metabolism in neuroblastoma. 1 Apr 50

Sixteen patients with abdominal neuroblastoma had 99m Technetium Phosphate Compounds (99m TC-PC) bone scans as a preoperative evaluation for metastatic disease. Ten patients (62%) had extraosseous tumor uptake while six patients (38%) did not. There was no difference in the incidence of tumor calcification, tumor necrosis or hydronephrosis in the two groups. However, VMA levels were significantly higher in the group with extraosseous tumor uptake. Various bone seeking radionuclides are compared to 99m TC-PC and possible mechanism for extraosseous uptake of such radionuclides are postulated. Awareness of the frequency of such uptake should reduce the possibility of errors in the interpretation of bone scans in patients with neuroblastoma.
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PMID:Extraosseous tumor uptake of 99m technetium phosphate compounds in children with abdominal neuroblastoma. 21 61

Serum samples from eleven children with neuroblastoma were drawn at diagnosis and again every 15 days or a month after the beginning of treatment over the course of 17 months observation. Twenty healthy children of the same age with no appreciable clinical manifestation were also studied as controls. Investigations included quantitative serum complement levels (total complement hemolytic activity, C3 and C4), anticomplementary serum activity and urine catecholamine levels (VMA and HVA). Complement levels of tumor patients were significantly higher than those of healthy subjects, but fluctuations were seen at different stages of the disease; patients at admission with active tumors or tumor recurrence had higher complement levels than those of patients in remission. Temporary recurrences of the disease were usually accompanied by fluctuations of urinary catecholamines, serum complement levels (mainly C3) and anticomplementary serum activity. At the terminal phase of the disease a drop in complement levels was usually seen, while urinary catecholamines were progressively increasing.
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PMID:Fluctuation of serum complement levels in children with neuroblastoma. 45 27

We describe the determination of the 4-O-methylated catecholamine metabolites 4-methoxy-3-hydroxyphenylacetic acid (iso-HVA), 4-methoxy-3-hydroxyphenylmandelic acid (iso-VMA) and 4-methoxy-3-hydroxyphenylethylene glycol (iso-MOPEG) in urine with use of mass fragmentography and deuterated internal standards. Normal values, expressed in terms of creatinine, are given as a function of age. Urinary excretion values during neuroblastoma, pheochromocytoma, and parkinsonism treated with L-DOPA, and during the intravenous administration of dopamine are discussed. For normal persons, the mean percentages (+/- SD) of 4-O-methylated metabolites relative to their 3-O-methylated analogs were 6.29 +/- 2.79% (iso-HVA/HVA), 0.70 +/- 0.57% (iso-VMA/VMA), and 0.77 +/- 0.53% (iso-MOPEG/MOPEG). We observed no significant changes in these parameters for the patients studied and conclude that the determination of 4-O-methylated catecholamine metabolites for these patients does not add information to the results obtained from their quantitatively more important 3-O-methylated analogs.
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PMID:Determination of 4-O-methylated catecholamine metabolites in urine by mass fragmentography. 47 19

We present a quantitative gas-chromatographic method for determining urinary 3-methoxy-4-hydroxymandelic acid (vanillylmandelic acid) and 3-methoxy-4-hydroxyphenylacetic acid (homovanillic acid). In this rapid technique an internal standard is used and the procedure involves ether extraction and silyl ether formation. Analytical recovery of vanillylmandelic acid averages 87.5% (CV, 0.95%), of homovanillic acid 102.3% (CV, 9.95%). Our data on 34 samples from 13 neuroblastoma patients show that homovanillic acid is more consistently elevated than is vanillylmandelic acid.
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PMID:Urinary 3-methoxy-4-hydroxyphenylacetic (homovanillic) and 3-methoxy-4-hydroxymandelic (vanillylmandelic) acids: gas-liquid chromatographic methods and experience with 13 cases of neuroblastoma. 92 75

By means of thin-layer chromatographic methods, iso-homovanillic acid (iso-HVA), iso-vanillactic acid (iso-VLA) and iso-vanilmandelic acid (iso-VMA) were determined in the urine of 10 children with neuroblastoma. The mean excretion of iso-HVA was 10.1% of total HVA excretion. Three patients excreted VLA; in their urine no iso-VLA was detected with certainty. All patients excreted high amounts of VMA; there was no detectable excretion of iso-VMA. Those results suggest that para-O-methylation could be limited to dopamine catabolism.
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PMID:Para-O-methylation of the catecholamines in neuroblastoma. 112 41

Since elevation of 3-methoxy-4-hydroxymandelic acid (VMA) excretion frequently occurs in patients with neuroblastoma, simpler methods have been developed to differentiate between high and low levels of VMA in the urine, namely the LaBrosse spot test and VMA test strips. Sensitivity of these two procedures depends upon the concentration of VMA in the urine rather than total amount of VMA excreted. VMA excretion data from patients with neuroblastoma show that even though the total amount of VMA excreted in a 24-hour period may definitely be elevated, the concentration of VMA in the 24-hour urine samples may vary greatly from one day to the next and from one patient to another. Therefore, these rapid screening methods for urinary VMA would appear to be too insensitive to use as the sole means of detection or follow-up of patients with neuroblastoma since these techniques require a high concentration of VMA.
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PMID:Screening procedures for neuroblastoma: false-negative results. 116 74

Quantitation of the urinary metabolites of catecholamines, including VMA, HVA, and metanephrines, from six normal preschool children was performed during a normal diet, a restricted diet, and a diet with increased amounts of vanilla, vanillin, and phenolic acids. Ingestion of these substances has been suspected of producing elevated values of urinary catecholamines and their metabolites. Urine was collected on the fourth day of each diet in two consecutive 12-hour aliquots, beginning at 8:00 AM. Statistically significant diurnal variation in excretion of all three metabolites and total free catecholamines (epinephrine, norepinephrine, and dopamine) was demonstrated. Diet did not alter exceretion of total free catecholamines or any of the three metabolites. This study suggests that a three-day restricted diet is not necessary prior to screening children for neuroblastoma when using quantitative assay methods and that all screening tests should be performed on a 24-hour urine sample.
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PMID:Effect of diet on urinary excretion of VMA, HVA, metanephrine, and total free catecholamine in normal preschool children. 124 41

The urinary excretion of catecholamines in 13 close relatives of two distantly related patients with congenital neuroblastoma was investigated. One of these relatives was a child with already known ganglioneuroblastoma. The catecholamines noradrenaline and dopamine were determined. Vanilmandelic acid and homovanillic acid also were determined and numerous other catecholamine metabolites were measured. In contrast to some previous reports in the literature, no pathological excretion patterns were found in the healthy family members.
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PMID:Excretion of catecholamines in relatives of patients with familial neuroblastoma. 124 89

Assays of urinary catecholamines and their metabolites (HVA, VMA, dopamine) permit biochemical diagnosis of neuroblastoma in approximately 80% of patients. The urinary methylated catecholamine metabolites normetanephrine (NMN), metanephrine (MN), and 3-methoxytyramine (3-MT) were analyzed in 18 patients with neuroblastoma and compared with reference values established for 69 healthy pediatric controls. All 18 neuroblastoma patients had raised urinary excretion of at least one of the three commonly assayed metabolites (HVA, VMA, dopamine). Similarly, raised urinary excretion of a methylated metabolite was noted in all but one of the neuroblastoma patients. The 3-MT level was pathologic in 16 of the 18 patients (89%). In this series, 3-MT assay sensitivity was sufficient to warrant trials on a larger population including comparison with patients considered nonsecretors by routine assay procedures.
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PMID:Methylated catecholamine metabolites for diagnosis of neuroblastoma. 157 31


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