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)

In two centres urinary excretion of HMMA was measured in a total of 359 children, aged from birth to 17 years, who were suspected of having a neuroblastoma; the diagnosis was subsequently confirmed in 39. Measurements were made on 24 h urine samples in 246 children, and on random samples in a further 113. The urinary excretion of HMMA relative to creatinine declined progressively with increasing age. After the age of four years the rate of decline was such that some age groups could be combined for statistical analysis. The range of values for each group was similar whether 24 h or random urine collections were used. It is concluded that the latter are adequate for the initial assessment of HMMA excretion.
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PMID:Urinary excretion of HMMA in children. 273 47

We report 2 cases of thoracic neural crest tumors complicating the course in patients with Beckwith-Wiedemann syndrome (BWS). In the first patient, a thoracic neuroblastoma was fortuitously discovered at age 3 months on a chest film prior to a partial glossectomy. In the follow-up left nephroblastoma and a right kidney simple cyst appeared. In the second patient, a thoracic tumor which proved to be a mature ganglioneuroma was discovered at age 4 years on a follow up spinal radiograph. Although less frequent than nephroblastoma and/or adrenal tumors, the occurrence of thoracic neuroblastoma in BWS suggests that periodic chest radiograph and assays of HVA, VMA and Dopamine should be included in the follow-up protocol.
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PMID:Beckwith-wiedemann syndrome and neural crest tumors. A report of two cases. 274 31

A 3-year-old girl was admitted to our hospital with a 3-month history of moderate fever, cough and dyspnea. Chest X-ray and C-T scan showed huge mass with high density, occupying right anterior thoracic cavity. Tumor marker including CEA, AFP, NSE in serum and VMA in urine revealed within normal data. Cytological finding obtained by percutaneous fine needle aspiration demonstrated neuroblastoma. 4 days after admission, urgent operation was performed, because of reinforced dyspnea. Huge tumor was successfully dissected, with combined resection of 2nd and 3rd ribs. Pathological diagnosis showed Ewing sarcoma originated from rib. This case is the youngest case of Ewing sarcoma of bone in Japan, and the patient is alive with disease free at the postoperative period of 21 months.
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PMID:[Ewing sarcoma originated from rib in 3-year-old child--a case report]. 276 50

In Sapporo City, the mass-screening for neuroblastoma concerning the urine of infants at about 6 months of age, started in 1981. After 1984, the quantitative determination of VMA, HVA in the urine was carried out using high performance liquid chromatography from the first step. About 104,000 babies have received the screening during the last 7 years, and 19 patients were found (incidence of detection 1:5,500). Except for one, who died of the duodenal perforation shortly after the surgery, all other patients are surviving free from disease. Among 10,000 neonates, we determined urinary concentration of VMA, HVA, and no positive cases were found. However, from this group, 2 patients we detected by the mass-screening at 6 months of age (1:5,000). After the start of mass-screening, the number of the patients with this tumor in much earlier stages and younger ages increased, clarifying that the main cause for improvement of the survival rates of neuroblastoma in Sapporo.
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PMID:Japanese experience of screening. 279 52

Twenty neuroblastoma and 4 nonneuroblastoma patients were studied by 131I-MIBG imaging. The primary tumor was detected in 89% of patients (8/9) before therapy. Bone marrow metastasis was also visualized in 4 of the 8 patients with primary positive scan. True negative results were obtained in 4 nonneuroblastoma patients. After therapy, of 10 tumor-bearing patients, eight showed positive scans and 9 of 12 lesions (75%) were visualized. The accuracies of presence or absence of neuroblastoma were compared between 131I-MIBG imaging and several tumor markers. The accuracies before and after therapy were as follows: 131I-MIBG imaging; 92% (12/13), 88% (15/17), serum NSE; 80% (4/5), 93% (13/14), serum LDH; 92% (11/12), 76% (13/17), urinary VMA; 54% (7/13), 56% (9/16), and urinary HVA; 77% (10/13), 56% (9/16). It appears that 131I-MIBG imaging is useful for both locating and excluding neuroblastoma. In addition, 131I-MIBG imaging appears to be the most efficient diagnostic and follow up study for neuroblastoma when it is combined with measurements of serum NSE.
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PMID:[Clinical evaluation of I-131 metaiodobenzylguanidine (MIBG) imaging in suspected neuroblastoma]. 281 Sep 11

Twenty two children with advanced retroperitoneal neuroblastoma and one child with advanced posterior mediastinal neuroblastoma admitted to our clinic were treated as follows. Seven patients (group A) underwent primary resection of tumor immediately after diagnosis. In two patients of this group, the levels of VMA and HVA in urine after surgery decreased to nearly normal (group A-I), while they did not change appreciably in the other 5 patients (group A-II). Seven patients (group B) underwent resection of tumor following complete or partial response to preoperative chemotherapy. Nine patients (group C) did not undergo resection of the tumor except for exploratory laparotomy. Two group A-I patients have survived, free of disease, for 6 months and 12 months after diagnosis. All patients of group A-II died within a year. Residual tumors of 4 patients of this group began to grow explosively just after surgery, although they received persistent postoperative chemotherapy. Four patients of group B survived for more than two years and the two patients of this group who received continuous intra-arterial PGE1 therapy had no postoperative explosive growth of residual tumors. Two patients in group C survived for 20 months and the others died within a year. Primary tumors and metastatic foci responded well to therapy as compared with group A-II, which suggests that presence of primary tumors may inhibit rapid growth of metastatic foci. Resection of primary tumors, therefore, was not always conducive to survival unless residual tumor responded to postoperative chemotherapy.
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PMID:An evaluation of surgical treatment and chemotherapy of advanced neuroblastoma (stage III & IV) with special reference to proliferation kinetics of residual tumors. 298 75

In the Saitama prefecture in Japan, VMA (vanillyl mandelic acid) screening spot test for detection of neuroblastoma has been performed in 173,046 infants in the years 1981-1986 and 15 infants were found to have neuroblastoma. Two infants had mediastinal tumors and the remainder 13 had intraabdominal tumors. Only 7 infants had palpable masses. Although CT was documented to be the best imaging procedure to provide sufficient information for treatment, conventional radiographic examinations of the chest and abdomen, and abdominal ultrasonography were able, as initial imaging procedures, to detect reasonably small neuroblastomas in infants with a positive VMA screening test.
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PMID:Imaging evaluation of infants with neuroblastoma detected by VMA screening spot test. 305 69

Measurements of urinary total catecholamines expressed as dopamine (TC) and their metabolites, total metadrenalines (TM) and 3-methoxy-4-hydroxy mandelic acid (HMMA) were made on 24-h urine collections from 56 previously untreated children with neuroblastoma. All results were expressed as a ratio to the creatinine excretion and were compared with "smoothed" age-related reference ranges derived from results in 704 children with other illnesses. Urinary catecholamines/metabolites excretion was elevated in 55 of the 56 patients, the exception being a baby with opsomyoclonus. TC was raised in 47, HMMA in 41, and TM in 37. Eleven patients had elevations of one parameter (TC in 10, HMMA in 1), 15 had 2 elevated levels, and 29 had elevations of all three. The TM level was the least contributory, as in no patient was it the only parameter elevated. Serial measurements were performed in 40 children, and the results correlated closely with the clinical progress of the disease and in some children permitted early detection of recurrence.
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PMID:Urinary catecholamines and their metabolites in management of neuroblastoma. 315 67

Serum ferritin is known to be one of the tumor markers for neuroblastoma. Serum ferritin is elevated in most children with neuroblastoma who are in stages III or IV, but it is not elevated in those in stage I or II. It has also been observed that iron load caused by blood transfusion shows a greater effect on serum ferritin levels than tumor activity due to neuroblastoma. Thus, serum ferritin increases in a linear fashion in children given repeated blood transfusions but the levels increase exponentially in children who have bulky neuroblastomas. Thus, serum ferritin values must be interpreted with caution in patients with advanced stages of neuroblastoma who inevitably require blood transfusions. The authors propose that the pretransfusion and posttransfusion serum ferritin values be in comparison with other tumor markers such as urinary VMA, urinary HVA, and neuron specific enolase. The serum ferritin level should not be used as a sole indicator of tumor activity in neuroblastoma.
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PMID:Serum ferritin in stage IV neuroblastoma. 318 16

The overproduction of catecholamines and their metabolites is a well recognised feature of neuroblastoma. Published data are scarce for their urinary excretion in children with neuroblastoma and in ill children in whom this diagnosis may be considered. We have determined a graphical upper reference limit for total catecholamines, total metadrenalines and HMMA in urine, expressed as a ratio to the creatinine concentration, for a group of 174 children with neuroblastoma and 704 hospitalised children with other disorders. This graph has been determined by examining the overlap region between the results for the two groups of children and avoids the irregularities caused by statistical outliers. The sensitivity and specificity of the individual tests indicate that total catecholamines is marginally the best single test to perform when trying to diagnose neuroblastoma, with the best clinical sensitivity being achieved by examining both total catecholamines and HMMA. Only two of the 174 children with neuroblastoma would not have been detected using these two tests. Total metadrenalines did not appear to add any further information and could be dropped from the repertoire in favour of the other two measurements.
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PMID:Neuroblastoma--when are urinary catecholamines and their metabolites 'normal'? 281 60


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