Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0677930 (primary tumor)
 20,210 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In 1973, mass screening program for 6-month old infants for early detection of neuroblastoma using a VMA spot test of a urine sample was initiated in Kyoto. In 1985, nation wide mass screening was initiated throughout the entire country and the Government has given the financial support to each district. In 1988, the Government recommended the institution of mass screening by quantitative measurements of VMA, HVA and creatinine using HPLC (high performance liquid chromatography), instead of the qualitative test of VMA alone. From 1974, at the time of initiation of mass screening for neuroblastoma to the end of October, 1989, 383 cases with this tumor have been discovered throughout the screening program. Three hundreds eighty three cases (88%) of them had been registered to the Neuroblastoma Committee of the Japanese Society of Pediatric Oncology. In this paper, the mass screening program was introduced and the 337 cases with this tumor detected by 6-month old screening were analyzed their clinical symptoms, findings, urinary VMA and HVA levels, primary sites, weights of primary tumor, histology, stages at diagnosis, metastatic sites, and the results of the treatment. Three hundreds twenty eight cases (97%) of them are expected to be cured. And we discussed clinical problems related to mass screening program for neuroblastoma, such as an increase of the incidence of infantile neuroblastomas detected by this program and the spontaneous regression.
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PMID:[Present status of neuroblastoma mass screening in Japan. Neuroblastoma-Committee of the Japanese Childhood Cancer Society]. 226 Aug 68

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

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

The purpose of the present study was to analyze the clinical pattern of neuroblastoma, the development of therapy, therapeutic results and the effect of neuroblastoma treated in childhood on the patient's later life. A retrospective series of 88 patient under 15 with primary neuroblastoma from years 1951-1978 was analyzed. The material was divided into two groups: patients admitted in 1951-1964 and those admitted in 1965-1978. Thirty three of the children (37.5%) were treated in 1951-1964 and 55 (62.5%) in 1965-1978. No statistical differences were seen between the therapeutic groups regarding age and sex distribution, site of the primary tumor and the histological and stage distribution. The mean age of all the patients on admission was 3.2 years (range 0-14.6 years). The age distribution was as follows: 24 (27.3%) 12 months, 18 (20.5%) 12-24 months and 46 (52.3%) over 24 months. There were 45 (51.1%) boys and 43 (48.9%) girls. All the histological specimens were reexamined. On the basis of histological differentiation, the series contained 67 (76.1%) neuroblastomas and 21 (23.9%) ganglioneuroblastomas. The localization of the primary tumors was the following: neck one (1.1%), mediastinum 17 (19.3%), elsewhere in the thorax 9 (10.2%), abdomen 15 (17.0%), pelvis 6 (8.8%), adrenal glands 31 (35.2%), dumbbell tumors 9 (10.2%). The stage distribution (Evans et al., 1971) was: stage I 6 (6.8%), stage II 20 (22.7%), stage III 36 (40.9%), stage IV 20 (22.7%) and stage IV-S 6 (6.8%). The diagnostic studies performed were chest roentgenography on 97.7%, skull roentgenography on 62.5%, pelvic roentgenography on 18.2%, spinal roentgenography on 23.8%, roentgenography of the long bones on 53.4%, and urography on 59.1% of the total. A bone marrow study was made on 36.4% of the cases. 24-hour urinary excretion of VMA was determined at the beginning of therapy for 78.2% and of HVA for 38.2% of the patients of the latter treatment group. The commonest general symptoms were anemia (34.1% of all patients), vague pyrexia (25.0%), lack of appetite, weight loss (18.2%) and poor general condition (15.9%). General symptoms were commonest in patients in stages IV and III (75.0 and 72.2%) and least frequent in stage I patients (16.7%). The average duration of the general symptoms in the survivors was 1.9 months and in the patients who died 1.7 months.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Neuroblastoma in 88 children. Clinical features, prognostic factors, results and late effects of therapy. 667 Aug 48

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

Neuroblastoma, a malignant tumor of infancy and childhood, has some very interesting peculiars: good prognosis, even with disseminated disease, propensity to occasionally undergo spontaneous regression, its ability to undergo spontaneous or induced differentiation to a benign ganglioneuroma. Neuroblastoma may originate anywhere along the sympathetic nervous system chain. The most common site of primary tumor is, however, within the abdomen either in the adrenal gland or in a paraspinal ganglions. A great deal of progress has been made in advancing the knowledge of human neuroblastoma at the cellular and molecular viewpoint. The genetic predisposition to develop the tumor is clarified, a specific oncogene amplified (N-myc) in neuroblastoma cells shows precise prognostic significance and the deletion of chromosome 1's short arm has been defined. Work-up in neuroblastoma's diagnosis include the urine assay for catecholamine metabolites (VMA, HAVA, VLA) and serum assay for the specific markers as neuron-specific enolase (NSE), ferritin, GD2 ganglioside. Imaging include CT-scan, MIGB body-scan and the newest monoclonal antibodies scan. Abdominal tumors are shown in about 75% of children > 12 months old. In 2/3 of cases, tumor is widely disseminated at the time of diagnosis. In the period 1979-94 the Italian Group for Neuroblastoma (GCN-AIEOP) collected 1083 cases of tumors and 5-yrs survival was 45% +/- 2.4 for the patients studied in the period 1979-84, which is increased to 58% +/- 3 for the group of patients 1990-94 (p < 0.001). The overall survival was 53 +/- 1.7. About 5-yrs survival at different stages, AIEOP shows that it is increased from 88% +/- 3.3 (1979-84 group) to 91% +/- 2.8 (1985-92) in the stage I and II (280 cases). In the stage IV survival value improved from 79% +/- 7.1 to 84% +/- 7 (132 cases). No statistical improvement can be observed, anyway. Better improvements can be pointed out in stage III (221 cases, survival from 48% +/- 5.2 (79-84 group) to 69% +/- 4.8 (85-92) and stage IV (483 cases, survival from 16% +/- 2.6 to 28% +/- 3.4) (p < 0.001). Finally we can summarize about neuroblastoma: 1) better prognosis in the first year of life; 2) ability to spontaneous regression, first of all, in stage IVs; 3) partial and provisional response to therapy in advanced stages; 4) no recovery increasing despite advancing in surgery and chemotherapy.
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PMID:[Recent advances on retroperitoneal neuroblastoma]. 941 95

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

Pheochromocytomas and paragangliomas are rare catecholamine-producing tumors which arise from chromaffin tissue. When a pheochromocytoma/paraganglioma is suspected, biochemical confirmation is based on 24-hour urinary excretion rates of catecholamines and their metabolites (metanephrines, VMA, etc.). Following biochemical confirmation non invasive imaging techniques such as CT and/or MR of the abdomen and 123I-MIBG scintigraphy are performed to localize the tumor. 111In-octreotide may also be applied, mainly to localize head and neck chemodectomas. Malignant paragangliomas of either adrenal or extra-adrenal origin show a variable natural history: from a locally invasive indolent tumor to a highly aggressive malignancy. Surgery with complete resection or debulking of the primary tumor is the standard treatment. External radiotherapy and chemotherapy are usually scarcely effective. An alternative treatment is 131I-MIBG therapy which is performed with high specific activity 131I-MIBG. Usually a standardized dose ranging from 3.7 to 9.1 GBq of 131I-MIBG is administered by slow i.v. infusion. In advanced stage cases 131I-MIBG therapy aims at symptom palliation and tumor function reduction as well as at tumor arrest or tumor regression. In these cases MIBG therapy allows prolonged survival and good quality of life. In less advanced cases the purpose of MIBG therapy is to complement surgery and to achieve the total eradication of the tumor. Non functioning malignant paraganglioma can some time also concentrate MIBG and can be treated with high doses of the tracer. 131I-MIBG therapy is a safe treatment and is usually well tolerated by the patient (with rather low myelotoxicity).
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PMID:Nuclear medicine therapy of pheochromocytoma and paraganglioma. 1073 85

The p73 gene is a p53 homologue which induces apoptosis and inhibits cell proliferation. Although p73 maps at 1p36.3 and is frequently deleted in neuroblastoma (NB), it does not act as a classic oncosuppressor gene. In developing sympathetic neurons of mice, p73 is predominantly expressed as a truncated anti-apoptotic isoform (DeltaNp73), which antagonizes both p53 and the full-length p73 protein (TAp73). This suggests that p73 may be part of a complex tumor-control mechanism. To determine the role of DeltaNp73 in NB we analyzed the pattern of expression of this gene in vivo and evaluated the prognostic significance of its expression. Our results indicate that DeltaNp73 expression is associated with reduced apoptosis in a NB tumor tissue. Expression of this variant in NB patients significantly correlates with age at diagnosis and VMA urinary excretion. Moreover it is strongly associated with reduced survival (HR=7.93; P<0.001) and progression-free survival (HR=5.3; P<0.001) and its role in predicting a poorer outcome is independent from age, primary tumor site, stage and MYCN amplification (OS: HR=5.24, P=0.012; PFS: HR=4.36, P=0.005). In conclusion our data seem to indicate that DeltaNp73 is a crucial gene in neuroblastoma pathogenesis.
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PMID:Expression of DeltaNp73 is a molecular marker for adverse outcome in neuroblastoma patients. 1185 3

Consorcial projects focused on 5 cancer types, breast-, colorectal-, head and neck- and pediatric cancers, and malignant melanoma. Breast cancer studies revealed unique splicing mechanisms concerning BRCA1. In sporadic breast cancers the involvement of DNA-repair genes was proved to be dependent on the histological type. Bone-metastatic tumors have been characterized by decreased NM23 and increased c-met and p53 expressions. C-erbB2 genotype of the primary tumor was not maintained frequently in bone metastases. Application of DNA-microarray and quantitative PCR technologies improved the prediction of therapeutic sensitivity of breast cancers. Colorectal cancer studies revealed regional inhomogenities (clusters) in various geographical regions of Hungary, which were distinct in the case of colonic and rectal cancers. To increase the sensitivity of fecal blood test of colorectal cancer screening, a new double-antibody test was developed and tested in a large cohort of patients. Genetic analysis revealed that hypermethylation is a significant factor in microsatellite instability which, and plays a role in silencing of APC and E-cadherin genes as well. The Hungarian pattern of TS polymorphism was also determined and was correlated not only with the efficacy of 5-FU treatment but with the progression of the disease as well. Population-based studies have been carried out in head and neck cancer patients (HNC) and smokers as well to reveal the genetic background of increasing tumor incidence. These studies revealed polymorphism in XRCC1/3 methylation enzyme gene which has preventive role. Other studies found frequent local immunosuppression in HNC patients. Studies indicated that the success of irradiation in this cancer type is dependent on the anti-vascular effects. Pediatric cancer studies determined the parameters of neuroblastoma screening based on VMA measurements. New splice variants of the WT1 gene involved in the monitoring of MRD of ALL patients was also described this year. We also obtained positive experimental data for the retinoic acid therapy of ALL. Melanoma studies extensively used DNA-microarray technology which identified 4 melanoma-specific and 2 melanoma progression-specific genes. In experimental human melanoma xenograft models we have identified 3 anti-metastatic agents: low molecular weight heparin, 2-methoxyestradiol and erythropoietin-alpha, where the later was characterized by specific effects on tumor vasculature.
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PMID:[Report of the National Oncology Research and Developement Consortium, 2003]. 1510


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