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)

The intracellular localization of tyrosine hydroxylase (TH), which is the rate limiting enzyme in catecholamine (CA) biosynthesis, and its activity in various adrenal and other neuroendocrine tumors was studied. TH was strongly localized in adrenal medulla, pheochromocytoma, and paraganglioma, but was scatteredly expressed in neuroblastoma. TH was not detected in adrenocortical tumors, ganglioneuroma, and other neuroendocrine tumors. Neuron specific enolase (NSE) was found in all neuroendocrine tumors, but Grimelius staining showed only the secreting granules of the tumor cells. TH activity was significantly high in pheochromocytoma and paraganglioma as compared with that in normal adrenal gland, whereas TH activity was low in a neuroblastoma and was undetectable in other tumors. These findings indicate that TH correlates well with the biosynthetic function of CA in the tumor cell and, thus, both the immunostaining of TH and the measurement of its activity in adreno-medullary and related tumors may provide some information about the process of cell differentiation in these tumors.
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PMID:Tyrosine hydroxylase indicates cell differentiation of catecholamine biosynthesis in neuroendocrine tumors. 752 77

Enolase catalyzes the interconversion of 2-phosphoglycelate and phosphoenolpyruvate in the glycolytic pathway. Enolase isozymes are dimers formed from three subunits (alpha, beta, and gamma). The gamma subunit, containing enolase (gamma-enolase) in serum, is important in the diagnosis of tumors originating from APUD (amine precursor uptake and decarboxylation) cells, like neuroblastoma, small cell carcinoma for the lung and certain carcinomas of the thyroid, the pancreas and the gut. Therefore, the determination of serum gamma-enolase levels is useful as a tumor marker of these neoplasms. On the other hand, raised gamma-enolase levels in cerebrospinal fluid have been demonstrated in patients suffering from various neurological disorders. In this paper, the author reviews the results of some of many studies related to gamma-enolase, up to now.
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PMID:[Gamma-enolase (gamma-eno)]. 760 91

A review of stage IV-S neuroblastoma is provided. The possible uses of prognostic features to guide treatment options in this group of infants with neuroblastoma are suggested. The biologic basis for the spontaneous regression of widespread tumor involvement in some infants with stage IV-S neuroblastoma is discussed. The reasons that some infants with IV-S disease progress to a fatal outcome, while most undergo maturation or involution and eventual long term cure are suggested. The influence of such factors as age at diagnosis, clinical staging, and tumor biology on eventual outcome are covered. Biological variables and markers discussed include: genetic (cytogenetics (1p deletions), nuclear genomic content), molecular biologic (N-myc oncogene amplification, mdr-1, ras, and trk, gene expression), immunological (major histocompatibility antigen density, cellular and humoral immunity), and biochemical (creatine kinase isoenzyme profile, neuron specific enolase, ferritin, chromatograffin, lactic acid dehydrogenase and catecholamine levels).
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PMID:Neuroblastoma stage IV-S. 763 37

Infants with neuroblastoma are known to have a favorable prognosis compared to those over 1 year of age. However, there is little biological information about the age-related heterogeneity of neuroblastoma. We evaluated the biological profile comparing cases detected by mass screening with those detected clinically. A total of 238 patients with neuroblastoma were classified into four groups according to their age at diagnosis. Patients in group A were 0-5 months of age (n = 31). Patients in group B were detected clinically and were 6-11 months of age (n = 25). Patients in group C were 6-11 months of age and were detected by mass-screening (n = 97). Patients in group D were more than 12 months of age (n = 85). The age-related heterogeneity was evaluated by Kaplan-Meier survival analysis, several clinical markers (neuron specific enolase, ferritin, vanillylmandelic acid and homovanillic acid) at diagnosis, tumor Ha-ras p21 expression and tumor N-myc amplification. Infant neuroblastoma had unique features in comparison to neuroblastoma diagnosed over 12 months of age. Clinical outcome of the patients in groups A and C was quite favorable. Even patients with stage III or IV disease in group A had a favorable prognosis. However, stage IVs disease in group A was not necessarily associated with a good prognosis and the early death after diagnosis was also characteristic. The biological profile of tumors in group C was similar to that in group A but different from the profile in groups B and D. Tumors in group B had a biological profile intermediate between groups A and D.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Age-related profile of neuroblastoma: a comparison of tumors detected by mass-screening with those detected clinically. 779 47

By means of a retrospective study made of multiple centres, it was aimed to determine which variables could be influential at the moment of diagnosis in the prognostic of patients suffering neuroblastoma and medullary bone affection. Fifty four cases of patients belonging to a total of ten hospital centers have been revised in a period of five years. The ages under study spanned from three months to eight years of age (mean = 2.83 years). For the analysis of the patients, they were divided into two groups: one being composed of the deceased patients and the other of the surviving. The deceased patients were of a more advanced age, the delay in the diagnosis was greater, the primitive tumor was found to be more frequently located in the adrenal glands, the metastasis appeared more readily in multiples, and the effectiveness of the treatment was less, resulting in lower cases of remission and a less radical surgery. The only difference with respect to other publications is that in the case of surviving patients, the number of cases of enolase and ferritin is more frequently pathological.
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PMID:[Neuroblastoma with bone marrow invasion. A multicenter study. Work Group of the Spanish Society of Pediatric Surgery]. 786 59

123I-meta-iodobenzylguanidine (123I-MIBG) scintigraphy was performed in 9 patients who were suspected to have a neuroblastoma. Positive images were obtained in 4 patients who had a neuroblastoma. Negative images were obtained in 5 patients who did not have a neuroblastoma. 123I-MIBG scan revealed an active tumor in a patient who had received intensive chemotherapy for the recurrence of neuroblastoma with returning of all tumor markers including neuron specific enolase to normal range. This indicated that 123I-MIBG scan can be used to assess the effect of therapy.
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PMID:[123I-MIBG scintigraphy in patients with neuroblastoma]. 802 23

Ewing's sarcoma is a very rare tumor which has, however, attracted much oncological interest since the dramatic improvement of its prognosis under chemotherapy. Its histogenesis has been discussed controversially for a long time, including a possible origin in immature reticulum, myogenous, endothelial and undifferentiated mesenchymal cells. Repeated reports have also suggested a possible neuroectodermal genesis. Convincing arguments, however, have only been brought forward during recent years, since it was found out that Ewing's sarcoma and malignant peripheral neuroectodermal tumor share a common chromosome translocation 11;22. In the meantime this hypothesis has been strengthened by numerous cell biological analyses. Histological differential diagnosis of Ewing's sarcoma has been improved by immunohistological methods. In most cases, they can be distinguished from lymphoma (leucocyte common antigen, B and T markers) and embryonal rhabdomyosarcoma (muscle specific actin, desmin). Apart from this, we now have an antibody specific for Ewing's sarcoma and malignant peripheral neuroectodermal tumors but not with neuroblastoma. Recent investigations regarding the prognosis under chemotherapy have shown that tumors with neural differentiation have a comparatively poor prognostic outcome. This is why in addition to the demonstration of Homer Wright pseudo-rosettes different neural markers such as neuron specific enolase, synaptophysin and chromogranin are to be analysed. Small cell osteosarcoma is a very rare sub-entity of osteosarcomas. Recent studies have shown that the tumor resembles Ewing's sarcoma with partial mesenchymal differentiation including osteoid formation.
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PMID:Round cell tumours of bone. 818 32

Neuroblastoma accounts for 24 of 109 patients who have been managed by the pediatric tumor outpatient clinic of our university hospital. Among the malignant solid tumors, neuroblastomas are the most numerous. We investigated neuroblastomas found by mass screening oncologically and epidemiologically. Up until March 31, 1991, seven cases were detected from 64,885 infants who received mass screening by the Kitakyushu City System which we had introduced in 1985. This system is based on an individual health survey program for infants in the city. Six of seven cases found by the screening were treated in our department. None of them, including stage III and stage IV cases, showed any conventional risk factors such as high serum levels of neuron specific enolase, ferritin, amplification of N-myc gene, nor cytogenetic abnormalities. Histopathological studies revealed that ganglioneuroblastoma was observed in 9 of 13 cases over one year of age, whereas it was observed only in two screened cases out of 11 cases under one year of age. According to the classification of Shimada et al., there was one stroma-rich tumor, which is rare in infants and usually a matured type, in the screened cases. Interestingly, another one of the six screened cases regressed spontaneously without any treatment. These cases suggested that some neuroblastomas in the process of maturation or spontaneous regression could be detected by mass screening. On the other hand, 9 of 13 non-screened cases over one-year-old died. Although mass screening at six months of age decreased the mortality rate by neuroblastoma in infancy, the prognosis of neuroblastoma in patients over one-year-old remained still poor. Mass screening should be carried out in a health survey program at one year and six months of age in order to improve the outcome.
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PMID:Different characteristics of neuroblastomas in cases found by mass screening and non-screening: evaluation of mass screening for neuroblastoma in Kitakyushu City. 826 87

The optimum time for a surgical resection of the primary lesion in the advanced neuroblastoma has not been defined properly. Recently the therapeutic results of the advanced neuroblastoma have improved through the use of preoperative chemotherapy. We check on our patients who were affected by this disease in the last 15 years. From 185 malignant tumours diagnosed in our department, 52 belonged to neuroblastomas with more than 12 months of evolution. Most of them were diagnosed during the year after their birth and it was not observed a difference of sexes. The location was the habitual one, except 2 cases of thoracoabdominal tumour and 1 of stesioneuroblastoma the most common manifestation was the metastatic disease. Catecholamines in 24 hours urine were positive in 74% of our patients, and enolase in 100% of the same ones. The longest percentage of patients is distributed in the advanced stages of Evans.
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PMID:[Neuroblastoma in childhood. Review of clinical aspects and of 2 forms of treatment]. 835 22

1. The mouse neuroblastoma cell line N-115 was used as a model system to study neuronal differentiation induced by treatment of cells with different agents. 2. The extent of morphological differentiation obtained with dibutyryl cyclic AMP (dbc-AMP), dimethyl sulfoxide (DMSO), retinoic acid (RA), and serum-free medium was correlated to the expression of the mRNA for the gamma isoform of the glycolytic enzyme enolase, a recognized neuron-specific marker. 3. A 4-day treatment of the cells with any of the differentiation inducing agents used in this study resulted in the extension of long neurites, though differences in cell body shape were observed depending on the agent used. 4. Northern blot analysis revealed that changes in the level of gamma enolase-specific mRNA correlate with the extent of morphological differentiation, with a 5- to 20-fold increase depending on the differentiation inducing agent used. 5. Finally, we found that a high cell density causes a significative increase in the level of the gamma enolase-specific message in cells maintained in growing conditions.
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PMID:Differential expression of neuron-specific enolase mRNA in mouse neuroblastoma cells in response to differentiation inducing agents. 839 14


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