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 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

Neuroblastoma is the commonest solid tumour in children under the age of 5 years (50% of cases before 2 years, 90% before 5) and the second cause of death after accidents. Approximately one child in 10,000 develops neuroblastoma by the age of 15 years. The situation in other European and North American countries is similar to that in France. As neuroblastoma is derived from the sympathetic nervous tissues, it is associated with the production of large amounts of catecholamines and their metabolites which are excreted in the urine. Less than 5% of cases do not produce catecholamines. Vanillylmandelic acid (VMA), homovanillic acid (HVA) and dopamine (DA) are the most useful chemical markers for the diagnosis and clinical control of neuroblastoma. They are generally measured using the reliable and sensitive high pressure liquid chromatography (HPLC). Survival is related to stage (the Evan's staging protocol has been superseded by the INNS staging), and age at diagnosis. There is almost 100% survival for stages I and IIa before the age of 12 months, and less than 20% for stage IV when diagnosed after 2 years of age. Multiple copies of the N-myc oncogene, deletions of chromosome 1p, and diploidy in tumour cells are associated with poor prognosis.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Neuroblastoma in children: clinical and biological aspects. An experience of screening in France]. 816 84

This study reports experience in the estimation of urinary catecholamines (uCATs) and their metabolites in the diagnosis and follow-up of neuroblastoma. Random urine samples were assayed for dopamine, noradrenaline and adrenaline, together with their metabolites 4-hydroxy 3-methoxymandelic acid (HMMA) and homovanillic acid (HVA), using HPLC with electrochemical detection. Twenty of 21 patients had elevation of one or more uCATs metabolites at diagnosis. Patients were monitored serially from diagnosis and, in those patients who had delayed resection of primary tumour (n = 13), particular attention was paid to levels at the pre-surgical evaluation as an indicator of persistence of viable disease at the time of surgery; dopamine proved to be the most accurate indicator of persistent disease at this time. Five of these patients developed recurrent disease, 4 of whom had elevation of two or more uCATs metabolites at the time of relapse. Several conclusions can be drawn from this study: (a) results for HMMA, HVA and dopamine in random urine samples will detect all but the most biochemically immature or inert tumours; (b) dopamine may be the most reliable indicator of persistent disease and (c) noradrenaline and adrenaline measurements were of little benefit. As results are expressed in relation to urinary creatinine, excretion of which may be affected by dietary protein and is therefore not constant, borderline results should be repeated.
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PMID:Interpretation of random urinary catecholamines and their metabolites in neuroblastoma. 819 81

Two cases of bilateral adrenal neuroblastoma in infants are reported. The first patient presented with abdominal distension due to metastatic hepatomegaly and the second patient was found because of elevated urinary VMA by VMA mass-screening system. Both were under one year of age and were classified as stage IVs neuroblastoma. In each case unilateral adrenalectomy was performed and the contra-lateral adrenal tumor was observed. The remaining tumors slowly decreased in size and clinical progress was good. A review of published cases of bilateral adrenal neuroblastoma suggests that the characteristics of this disease are age less than 1 year, stage IVs, and good prognosis.
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PMID:Bilateral adrenal neuroblastoma. 819 31

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

A case of stage 4 neuroblastoma that developed excessive hypertension on day 120 of chemotherapy is presented. The tumor initially had responded well to chemotherapy; however, while the tumor mass decreased, plasma and urine catecholamines and the blood pressure increased. The plasma concentrations of noradrenaline, adrenaline, and dopamine increased to 26.4, 1.8, and 36.2 micrograms/l, respectively. The profile of catecholamine metabolites changed: on day 150 of therapy, noradrenaline, adrenaline, and dopamine levels were increased, whereas HVA and VMA levels were decreased when compared to day 1 of therapy. The only residual neuroblastoma tissue visible on MIBG scintigraphy on day 150 of treatment was a metastasis in the left tibia which was irradiated with 24 Gy. The adrenaline concentration in the left femoral vein was twice as high compared to the right femoral vein. A treatment, possibly radiation-associated tumor cell alteration resulting in a different catecholamine production, is discussed.
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PMID:Development of hypertension in neuroblastoma during therapy: a case report. 851 30

Between April 1985 and March 1994 (9 years), 229,346 babies were born in Niigata prefecture, and 201,888 (88.0%) underwent mass screening (MS) for neuroblastoma at 6 months of age. To date, 29 infants have been screen-detected as having neuroblastoma (1:7908). All screen-detected patients survived after removal of the primary tumor. In the same birth cohort, 17 additional children were clinically diagnosed as having neuroblastoma. The cumulative incidence rate of neuroblastoma at 5 years of age was 10.5 per 100,000 live births in the 5-year birth cohort before MS was introduced, and 18.6 per 100,000 in the first 5-year birth cohort after MS was introduced. These values were not statistically different. The birth cohort incidence rate increased significantly to 22.2 per 100,000 (p < 5% compared with before MS) after the method of MS was changed to high-power liquid chromatography (HPLC), even though this latest birth cohort has not been followed for 5 years. The population-based mortality rate from neuroblastoma was 5.9 per 100,000 in the 5-year birth cohort before MS, and 4.5 per 100,000 after MS in the first 5-year birth cohort, using the vanillymandelic acid (VMA) spot test. These values were not statistically significant. In contrast, no death was observed in the next 4-year birth cohort after MS using HPLC; however, this birth cohort has not yet been followed for 5 years.
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PMID:Screening for neuroblastoma: a 9-year birth cohort-based study in Niigata, Japan. 856 31

To detect neuroblastoma in early stages, mass-screening of this tumor was carried out by HPLC method using the urine of six-month-old infants. Screening began in April, 1981, and a total of 42 cases were detected by December, 1994. The incidence of detection was about 1:5,000 babies tested. All of these patients have survived without relapses except one, who died of surgical complications. Nevertheless, tumors developed at later stages in 14 cases from the group which resulted negative at the 6-month screening. To determine whether such tumors could be detected by a second check, a 14-month screening was carried out, beginning in April, 1991. By December, 1994, 3 patients with neuroblastoma were found among 41,809 babies tested, and were subsequently treated. While one of these three patients was not screened at 6 months, the other two had shown approximate cut-off values in urinary VMA as well as HVA at their 6-month screening. Therefore, if the tumors had already existed at the 6-month screening, and had grown gradually until the second check at 14 months, at least some of these tumors were of the late-onset variety and could have been detected at an earlier stage.
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PMID:Mass-screening of neuroblastoma using urine from infants by high-performance liquid chromatographic method: results of first (6th month) and second (14th month) screening. 904 45


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