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)

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

Rat models of Parkinson's disease typically employ a rapid nigral injection of 6-hydroxydopamine (6-OHDA) to produce a near-complete loss of nigrostriatal dopamine neurons, and thus, model end stage disease. The present report describes the use of a continuous, low dose infusion of 6-OHDA into the striatum which produces a terminal axotomy of nigrostriatal dopamine neurons and protracted behavioral response. A solution of 6-OHDA in 0.4% ascorbate, delivered at 37 degrees C from osmotic minipumps, was stable for 8 days as determined by its retained toxicity to a dopaminergic neuroblastoma cell line. The continuous infusion of 0.2 mu g 6-OHDA per h did not affect the striatal uptake of [3H]%GABA, [3H]choline, or [3H]glutamate but reduced [3H]dopamine uptake by 55% within 1.5 days after the start of the infusion. The striatal infusion of 6-OHDA produced a dose-dependent reduction of striatal dopamine and DOPAC levels but did not alter HVA, 5-HT, or 5-HIAA. An increase in amphetamine-induced ipsiversive rotations occurred within 1.5 days after the acute striatal injection of 20 mu g or 30 mu g of 6-OHDA but required 4 days to develop with the continuous 6-OHDA infusion. The topography of the lesion mapped by [3H]mazindol binding showed that, beginning by 1.5 days, a diffuse depletion of terminals encompassed much of the striatum in the 30 mu g acute injection group, whereas in the continuously infused rats, the lesion was apparent only by 4 days and was restricted to a smaller and more completely lesioned area. Unlike acutely lesioned animals, continuously infused rats revealed no obvious loss of dopamine neurons in the pars compacta by 5 weeks after 6-OHDA. The continuous striatal infusion of 6-OHDA can produce a topographically limited terminal axotomy of dopamine neurons and a protracted behavioral impairment.
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PMID:A continuous striatal infusion of 6-hydroxydopamine produces a terminal axotomy and delayed behavioral effects. 883 64

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

A 3-year-old girl presented with epigastric pain and physical examination showed a hard right upper quadrant mass. Urine VMA and HVA were found to be raised. CT scan showed a large mass arising from the right adrenal gland, with necrotic areas and ring calcification There was midline extension. The diagnosis of neuroblastoma was confirmed by bone marrow aspiration biopsy. As the patient had inoperable stage IV neuroblastoma, she was treated with multi-drug chemotherapy. Follow-up CT scan showed excellent response to the chemotherapy, and the tumour was successfully resected. Harvesting of autologous peripheral bone marrow stem cells, megatherapy with Melphalan, and marrow rescue with the stem cells were effective. The child has been well for three years to date. The clinical and imaging features of neuroblastoma, particularly the role of imaging in staging, are emphasised.
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PMID:Clinics in diagnostic imaging (31). Adrenal neuroblastoma. 955 Sep 15

Iodine-123-MIBG (123I-MIBG) scintigraphy were performed for 23 patients with neuroblastoma at diagnosis. The intensity of MIBG activity in the primary tumor was evaluated visually (grade 3; intense uptake-grade 0; no definite uptake), and its relationship to the size, degree of tumor spread, urinary catecholamine metabolites (VMA, HVA), and histological types were investigated. The results of 123I-MIBG uptake grade were as follows: grade 3; 44% (10/23), grade 2; 30% (7/23), grade 1; 17% (4/23), grade 0; 9% (2/23). The grade was not associated with the tumor size, or the degree of tumor extension to the distant lesion, either. The more catecholamine metabolites were excreted in the urine, the tumor tended to have more intense uptake. The tumors of neuroblastoma rosette fibrillary type, and ganglioneuroblastoma poorly differentiated type had more intense uptake than neuroblastoma round cell type and ganglioneuroblastoma well differentiated type. The case of ganglioneuroma did not have definite MIBG uptake. The intensity of MIBG uptake is not relevant to the pathological grade of neuroblastoma, but considering the electromicroscopical features of neuroblastoma reported previously, it is thought to reflect the histological type.
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PMID:[Iodine-123-MIBG scintigraphy in neuroblastoma; relationship between the intensity of uptake and tumor characteristics]. 1058 43

Pheochromocytoma and neuroblastoma are distinct tumours, but their biological diagnosis is based on secretion increase of one or several catecholamines. Assays have to be very sensible and specific for an early diagnosis. 24 hours urinary catecholamines and metabolites are currently measured, but technical improvements permit plasma metanephrine assay, an excellent indicator of pheochromocytoma. HPLC coupled to electrochemical detection represents the most efficient methodology. After a review of urinary and plasma assay methods, the authors show usual values of catecholamines, metanephrines, HVA and VMA, according to ages, and give examples of results encountered in classical or not tumours and in falsely positive cases. Urinary metanephrine assay is the most sensible and specific in biological diagnosis of pheochromocytoma, while catecholamines and VMA assays lack of sensibility. Results have to be given by 24 hours and by creatinine ratio. Metanephrine assay can be performed also in plasma and exhibits the same interest. However, in urine as in plasma, in case of renal failure, results cannot be interpreted. Neuroblastoma biological diagnosis is based classically on HVA, VMA, and dopamine assays, nowadays only in 24 hours urine (or in urinary micturition for screening), and results are also expressed as creatinine ratio. But even if several assays are advisable, 5% of the neuroblastoma cases do not produce increased catecholamine values. In some cases, metanephrine assay could be of interest. After the age of 12 months, clinical expression of neuroblastoma is dramatic in 70% of cases. So, a biological screening has been experimented in several countries including France. A French translation of the consensus conference report (1998) is appended, which shows the complexity of neuroblastoma screening. Now, there is no evidence that early tumour detection by screening lessens the mortality rate, but a weak benefit is not excluded.
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PMID:[Biochemical diagnosis of pheochromocytoma and neuroblastomas]. 1183 Mar 91

Overproduction of catecholamines (dopamine [DA], norepinephrine [NE]) and their metabolites (homovanillic [HVA] and vanillylmandelic [VMA] acids) characterizes neuroblastoma (NB). In previous studies, increased urinary DA/NE, and DA/VMA ratios have been associated with poor prognosis, whereas low DA/NE ratios have been associated with longer disease-free survival. Higher urinary VMA, HVA, and NE levels have been found in association with low MYCN amplification, in contrast to cases with high MYCN amplification in which normal levels have been found. It is then believed that an "immature" catecholamine pattern indicates poor prognosis. We correlated urinary DA, NE, VMA, and HVA levels with age, clinical tumor stage, histological features (favorable [FH]/unfavorable [UH]) and MYCN status of 33 patients with NB. DA/VMA and DA/HVA ratios were also calculated. Wilcoxon rank sum and chi-squared tests were performed to determine statistical significance. Eighty-eight percent (15/17) of stage 3-4 cases had DA levels >2 times the upper limit of normal, but only 8% (1/12) of stage 1-2 cases had DA levels twice the upper limit of normal. In 61% (11/18) of stage 3-4 cases, the VMA level was >10 times the upper limit of normal, in contrast to stage 1-2 cases, in which only one patient (1/15) had a VMA level >10 times the upper limit of normal. Similar findings were obtained with urinary HVA and NE. Patients older than 12 months of age at diagnosis also had higher urinary levels of DA, VMA, HVA, and NE than those of patients younger than 12 months of age at diagnosis. Eighty-two percent (14/17) of stage 3-4 cases had DA/VMA ratios <0.78, with the other 18% (3/17) showing ratios between 1.4 and 8.82 (all stage 4 and >12 months of age). In contrast, all stage 1-2 cases ((12)) had ratios <1.4. All (12/12) non- MYCN-amplified cases had DA/VMA ratios <1.4 (0.06-0.84), while one MYCN-amplified case (1/3) had a ratio of 8.82; the other two MYCN-amplified cases had DA/VMA ratios of 0.09-0.11. Twenty-nine percent (2/7) of cases with UH had a DA/VMA ratio >1.4, but in all FH cases (14/14) the DA/VMA ratio was <1.4 (0.08-0.084). Similar to previous studies, we found that aggressive NB is associated with higher urinary levels of DA, VMA, HVA, and NE. We also confirmed the previous observation that there appears to be a subset of NB in which a possible blockade in DA metabolism is associated with poor prognostic features (>12 months, stage 4, UH, and MYCN amplification). A seemingly novel observation in our study is that all high DA/HVA and DA/VMA ratios were obtained in stage 4 tumors, suggesting an association between the inability to metabolize DA and the acquisition of metastatic potential. On the basis of our results, we would like to emphasize the importance of determining not only DA, HVA, and VMA urinary levels, to support the diagnosis of NB, but also DA/HVA and DA/VMA ratios as a rapid initial assessment of prognosis in these patients.
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PMID:Hormonal activity may predict aggressive behavior in neuroblastoma. 1191 May 15

Dopa-responsive dystonia (DRD) is an extrapyramidal disorder caused by deficit of 5,6,7,8-tetrahydrobiopterin (BH4), cofactor for tyrosine hydroxylase (TH). In these patients the nigrostriatal dopaminergic neurons normally express TH and the cellular machinery for the dopamine uptake. LA-N-1 is a human neuroblastoma cell line expressing tyrosine hydroxylase. Here we show that LA-N-1 cells are able to take up exogenous dopamine (DA) by an high-affinity mechanism; significant amounts of serotonin and its metabolite 5HIAA, but neither DA nor its metabolites, DOPAC and HVA, could be measured in the cell culture homogenate. 5,6,7,8-Tetrahydrobiopterin, cofactor for both tyrosine and tryptophan hydroxylases, is able to activate dopamine synthesis and also decreases the content of 5HIAA by 50%, indicating that LA-N-1 might be a useful model for studying dopamine-serotonin interaction in cultured cells and the neuronal mechanism of DRD.
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PMID:6R-Tetrahydrobiopterin induces dopamine synthesis in a human neuroblastoma cell line, LA-N-1. A cellular model of DOPA-responsive dystonia. 1210 Oct 48

Neonatal adrenal abscess is an extremely rare condition. 32 cases, 4 bilateral, have been described in the world literature. We treated successfully other two bilateral cases. We report on this rare condition and review the world literature. In our Department we observed two patients in the neonatal period with abdominal distension, vomiting, irritability and fever. Abdominal ultrasound (US), plain x-ray of the abdomen, intravenous pyelography and computed tomography (CT) of the abdomen were performed. In both cases bilateral suprarenal cystic masses were identified. Vanilmandelic acid, Homovanillic acid and cathecolamines were normal. The two neonates underwent a surgical exploration. Abscesses were found and drained releasing a moderate amount of haemorrhagic and purulent materials from the adrenal glands. Post-operative histology on the surgical specimen showed in both cases an abscess in partial haemorrhagic adrenal glands. No neoplastic cells were observed. The recovery was uneventful and at six months follow-up both patients were well and without signs of adrenal insufficiency. Haematogenous bacteria seeding a normal gland or abscess formation in a previous haemorrhagic gland are the most accredited theories. Neuroblastoma, Wilm's tumor and renal duplication with dilatation of the upper segment must be considered in the differential diagnosis. Percutaneous drainage (+/- biopsy) under CT or US guide should be considered the treatment of choice, followed by surgical exploration when diagnosis is not clear.
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PMID:Bilateral neonatal adrenal abscess. Report of two cases and review of the literature. 1460 Dec 36

The case of a left adrenal mass detected by ultrasonography in a male fetus at the 37th week of gestation is presented. After an uneventful delivery at the 38th gw, the newborn was sonographically monitored, and during the follow-up clinical evaluations were performed, in order to make a differential diagnosis between an adrenal hemorrhage and a congenital neuroblastoma. VMA and HVA were within normal levels, and MIGB scan was negative. A MNR of the the abdomen performed at the age of one month was suggestive for an adrenal hemorrhage in involution. We therefore decided to keep monitoring the mass by ultrasound, avoiding any surgical approach. The sonographic complete disappearance of the disease was documented at the end of the fourth month.
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PMID:[Spontaneous regression of an adrenal mass detected in utero]. 1505 43


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