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 girl with congenital extraadrenal neuroblastoma died at 3 months of age with multiple parenchymal metastases in the brain and spinal cord. The bones of the skull, cranial dura, venous sinuses, and orbits were not involved. Autopsy findings suggest that the tumor penetrated the spinal meninges and disseminated through the cerebrospinal fluid. It is possible that hematogenous metastasis occurred as well. The intracerebral metastases were not identified on a CT scan performed several hours before the child's death.
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PMID:Retroperitoneal neuroblastoma widely metastatic to the central nervous system. 42 83

The clinical manifestations of 253 neuroblastoma cases in childhood, treated in Denmark from 1943 to 1980, were reviewed. Most striking was the vagueness of symptoms in the majority of patients, only a few of whom exhibited the symptoms strongly suggestive of a neuroblastoma (i.e. the Horner syndrome, the watery diarrhoea syndrome, the dancing eye syndrome). The vagueness of the symptoms might have led to fatal procrastination of the diagnosis. The diagnostic delay has, however, no independent prognostic significance for survival in our patients (p = 0.09). The maximal tumour spread was recorded for all 253 patients, and the distribution of metastases was in accordance with the "soil-seed" hypothesis. The tumour spreads with equal frequency by local growth, by lymphatic vessels to distant lymph nodes, and by blood to bone. Only in widely disseminated tumours are metastases to the lungs, the meninges, the brain, and the reproductive organs seen to occur. Eighty-five percent of the patients, for whom data were available, excreted VMA above the normal value for their age, and 43% excreted Norepinephrine + Epinephrine (N + E) above normal levels. The excretion of both VMA and N + E was significantly correlated to stage, and thus to prognosis. Neither the level of VMA excretion nor the level of N + E had any bearing on the survival when age and stage were adjusted for. Serial VMA and N + E determinations show that patients with normal values for these parameters had significantly better prognosis than patients with elevated values during the first, second, third and fourth trimesters after the initiation of treatment. Increasing values in the individual patient were associated with a poor prognosis. We found no correlation between the initial leucocyte count and survival when age and stage were adjusted for.
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PMID:Signs, symptoms, metastatic spread and metabolic behavior of neuroblastomas treated in Denmark during the period 1943-1980. 363 7

Critical Evaluation of 200 tumours of meninges, brain and spinal cord showed that to be familiar with the ultrastructural features of meningioma and its variants was instrumental in differential diagnosis of other primary or secondary meningeal tumours (neurinoma, paraganglioma, xanthomatous and histiocytic tumours). A limited value of electron microscopy was found in astrocytoma and glioblastoma in contrast to its importance in low-differentiated ependymoma and oligodendroglioma. The examination had histogenetical and taxonomic values in medulloblastoma (CNS neuroblastoma and mixed tumours with a component featuring primitive neuroectodermal or neuroblastic differentiation). Ultrastructure was very important in the so-called primitive neuroectodermal CNS tumours where only the lack of conspicuous glial or neuroblastic differentiation confirmed the diagnosis. Electron microscopy was instrumental in rare primary CNS lymphomas as well as in some metastatic tumours.
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PMID:[Contribution of electron microscopy in the differential diagnosis of tumors of the meninges, brain and spinal cord]. 373 Dec 97

Review of the literature reveals much disparity regarding the incidence, natural history, and survival rate in patients harboring esthesioneuroblastoma (olfactory neuroblastoma). The metastatic tendencies of this tumor have been highly variable in the literature, and reported intracranial involvement has been via local extension from the tumor's origin near the cribriform plate. Our present case describes a metastasis of esthesioneuroblastoma to the right parietal cortex and meninges in a patient without evidence of local recurrence after combined surgical and radiation therapy 5 years previously. The broad spectrum of biological behavior of this tumor, and its response to various therapies are discussed. The immunohistochemical and ultrastructural features of this unusual tumor are also presented. Recommendations are made for the management of these patients, including regular serial neurological examinations, serial computed axial tomography or magnetic resonance imaging of the head and neck, and, in the initial management, chemotherapy.
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PMID:Late intracranial metastasis from esthesioneuroblastoma: case report and review of the literature. 378 1

A case of olfactory neuroblastoma in a 9 month-old girl is reported. Blindness and purulent meningitis were the first symptoms. Radiological examinations including CT scan revealed a sphenoidal tumor with penetration of the meninges. Surgery revealed an ethmoido-sphenoidal tumor mass extending into the pituitary fossea which consisted of malignant cells with the histological aspect of esthesioneuroblastoma. The rarity of this tumor in early childhood and its clinical polymorphism are emphasized.
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PMID:[Olfactory neuroblastoma disclosed by blindness in a 9-month-old girl]. 400 68

Cefotiam (CTM) was evaluated for its safety and efficacy in children. Twenty-six patients were treated with 40 to 200 mg/kg per day of CTM by intravenous administrations. The diagnosis of the patients were acute pharyngitis (2), acute bronchitis (1), pneumonia (4), empyema (2), urinary tract infection (2), typhoid fever (1), acute enterocolitis (2), partially-treated purulent meningitis (1), and suspected septicemia in neuroblastoma (1); and the remaining ten patients were considered to have nonbacterial infections. The pathogens recovered were Streptococcus pyogenes (1), Streptococcus pneumoniae (1), Staphylococcus aureus (4), Haemophilus influenzae (4), Escherichia coli (1), enteropathogenic Escherichia coli (1), Salmonella typhi (1), and Campylobacter jejuni (1). All but two patients of bacterial infections were cured after the CTM therapy, and the rate of efficacy was 87.5%. Diarrhea (3), urticaria (1), transient elevation of GOT and GPT (1), and transient eosinophilia (3) were found to be associated with the CTM therapy. However, no severe adverse reactions were encountered. Half life of the serum CTM level was 0.93 +/- 0.13 hours, and excretion into the urine was rapid. CSF concentration obtained 1 hour after an intravenous injection of 21 mg/kg of CTM in a case with inflamed meninges was 1.5 mcg/ml, and the CSF/serum ratio was 9.0%. From these data, CTM appears to be a safe and effective antibiotic when used in children with susceptible bacterial infections.
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PMID:[Clinical evaluation of cefotiam therapy in children (author's transl)]. 627 Apr 13

Metastasis to the brain or spinal cord parenchyma is extremely rare in cases of neuroblastoma. We present a 3-year-7-month-old boy with neuroblastoma, stage IV, with intraspinal metastasis. He had no neurologic manifestation except incontinentia urinae and ataxia at the terminal stage. His cranial computed tomography scan showed high density areas in both cerebellar hemispheres which seemed to be distant metastatic masses. At autopsy, the metastases in the cerebellum were found to be due to dural infiltration but in the spinal cord parenchyma of the lumbar spine metastases were detected macroscopically. There were multiple distant metastatic areas near the roots, anterior and posterior. The neuroblastoma seemed to have spread along the roots by direct invasion from the meninges. In the future, the number of patients with metastasis into the brain or spinal cord parenchyma will increase because patients with progressive disease could live for a long time as a result of intensive chemotherapy. Observation of these cases will be helpful to clarify the routes of metastasis to these areas.
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PMID:Intraspinal metastasis of neuroblastoma--report of a case detected at autopsy. 664 87

Mouse N18TG2 neuroblastoma and rat C6 glioma cell lines were injected into male nude mice, and the tumors were passaged serially. At each generation, tumors were analyzed for delta opioid binding using [3H][D-Ala2,D-Leu5]enkephalin and for sigma 1 and sigma 2 binding with 1,3-[3H]di-o-tolylguanidine in the presence and absence of 1 microM pentazocine. Receptor density (Bmax) and affinity (KD) were estimated by homologous competition binding assays. Opioid and sigma Bmax values in the solid tumors were significantly lower than their original levels in vitro. KD values for opioid/sigma ligands were similar in vitro and in vivo. With successive passages in the murine host, delta opioid and sigma 1 binding of the neuroblastoma-derived solid tumors became undetectable. In contrast, sigma 2 receptor Bmax values were unchanged with successive passages of the neuroblastoma-derived tumors and doubled in the nude mouse-borne gliomas. When neuroblastoma-derived solid tumors that were devoid of delta opioid binding were returned to culture, opioid receptors appeared to be up-regulated as compared with their original in vitro levels. Serial passaging of these recultured cells in vivo again resulted in a rapid decline in opioid receptor content. The opioid data are consistent with our prior findings on opioid binding diminution in human brain tumors. The pattern of change for sigma binding was more complex, with the sigma 2 response in late passages of the glioma being reminiscent of the formerly observed increase in number of sigma sites in transformed human meninges, kidney, and colon tissue.
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PMID:In vitro and in vivo expression of opioid and sigma receptors in rat C6 glioma and mouse N18TG2 neuroblastoma cells. 803 81

A 19-year-old female presented with an unusual intracranial extracerebral neuroblastoma originating from the dura mater. The tumor was located in the right frontal convexity, and had adhered neither to the underlining arachnoid membrane nor the cerebral cortex. The tumor feeders were meningeal arteries. Light microscopy showed the tumor consisted of small compact cells, forming numerous Homer-Wright rosettes. Immunohistochemical examination and electron micrography confirmed the diagnosis of neuroblastoma. Intracranial extracerebral neuroblastoma is extremely rare, especially originating from the dura mater. This neuroblastoma probably originated from heterotopic neuroglial nest cells in the meninges.
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PMID:Intracranial extracerebral neuroblastoma originating from the convexity dura mater. 878 30

Laminin-1 is a glycoprotein found in the basement membrane of many tissues. In the cerebellum of rodents, it has also been localized along Bergmann glial fibers, where it is thought to be involved in promoting granule cell migration by enhancing adhesion and neurite outgrowth along these fibers. Recent reports, however, indicate that laminin-1 is not present on Bergmann fibers, but instead is associated with blood vessels and meninges. Furthermore, attempts to block granule cell migration using antibodies against laminin-1 have yielded conflicting results. In this report, we provide further evidence that laminin-1 is associated exclusively with blood vessels and meninges in the cerebellum of postnatal rats. In addition, we show that adhesion and neurite outgrowth of granule cells was impeded on laminin-coated surfaces. In fact, cerebellar cells dramatically and consistently avoided laminin-1 regions of patterned surfaces. Cells did adhere to laminin regions if it was coadsorbed with polylysine or tested in serum-containing medium. Avoidance of laminin-1 regions in culture was not, however, blocked by pretreatment with laminin-1 antibodies. By comparison, mouse neuroblastoma cells adhered preferentially to laminin-1 regions in serum-free medium, a response which was blocked by laminin-1 antibodies. These results indicate that laminin-1 is not involved in granule cell migration along Bergmann glial fibers. Instead, they suggest that laminin-1 may function as a repulsive guidance cue preventing granule cells from following inappropriate pathways during development.
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PMID:Is laminin-1 a guidance cue for cerebellar granule cell migration? 921 71


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