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 49-year-old man had a malignant soft tissue tumor of the right thigh with metastasis to the femoral region and lower quadrant of the anterior abdominal wall on the right side and the left supraclavicular lymph nodes. The neoplasm showed features of chondrosarcoma and primitive neuroectodermal tumor (combined neuroblastoma, ependymoma, astrocytoma, and oligodendroglioma). The gliomatous part of the mixed tumor was confirmed by identification of the glial fibrillary acidic protein (GFAP). The diverse cellular population suggests a tumor origin from the ectomesenchymal remnant of the neural crest. The mesenchymal component of the neural crest would differentiate into the chondrosarcoma and the neuroectodermal component into the primitive neuroectodermal neoplasm. These various neoplastic elements, then, would form a neoplasm of mixed mesenchymal and neuroepithelial origin or an ectomesenchymoma.
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PMID:Malignant neoplasm of mixed mesenchymal and neuroepithelial origin (ectomesenchymoma) of thigh. 649 17

In normal conditions, neuron-specific enolase (NSE) is histochemically demonstrable only in neurons and cells of the amine precursor uptake and decarboxylation (APUD) system. This has been found not to be true for neoplastic cells. Several types of CNS tumors, including glioblastoma, astrocytoma, oligodendroglioma, ependymoma, medulloblastoma, pineocytoma , meningioma, and choroid plexus papilloma, focally stained positively for NSE. Reactive astrocytes were also frequently positive. In the peripheral nervous system, neuroblastoma, ganglioneuroma, and paraganglioma stained positively for NSE. A number of non-APUD tumors were focally positive. These included schwannoma, carcinoma and fibroadenoma of the breast, renal cell carcinoma, giant cell tumor of the tendon sheath, and chordoma. Caution should be exercised in relying on the immunohistochemical demonstration of NSE as a diagnostic marker in those tumors that do not belong to the APUD cell system. It seems of little value as evidence of differentiation in CNS tumors.
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PMID:Immunohistochemical demonstration of neuron-specific enolase in neoplasms of the CNS and other tissues. 654 18

A rare case of cerebral neuroblastoma with extracranial metastases is reported. This patient was followed for over ten years. The biopsy specimen of the brain tumor taken at the first operation revealed the architecture of a poorly differentiated ependymoma having perivascular rosettes. The histological pattern of the second biopsy taken eight years after the first operation was highly cellular and vascular with abundant mitoses, showing Homer-Wright rosettes. The histological diagnosis of this second specimen was a cerebral neuroblastoma. The problem of the multipotentiality of the brain tumor is discussed.
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PMID:A cerebral neuroblastoma with extracranial metastases. 728 Sep 73

Analysis of the CT findings in 35 cases of tumoral hemorrhage (taken from 973 intracranial tumors) revealed three distinct patterns of bleeding: (a) hematoma, (b) central hemorrhage, and (c) hemorrhagic infarction. The location, multiplicity of lesions, and contrast enhancement are important in the diagnosis, and the clinical history and arteriography may also be helpful. The largest single group in this series consisted of 12 metastatic lesions: the others included glioblastoma (7), chromophobe adenoma (4), Grade I astrocytoma (3), medulloblastoma (3), central neuroblastoma (2), histiocytic lymphoma (2), and ependymoma (1). The relatively low mortality rate (21/35) despite marked neurological deterioration is attributed to prompt CT demonstration of hemorrhage followed by aggressive therapy (surgical evacuation, total resection, radiotherapy, and/or steroids or mannitol).
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PMID:Computed tomography of acute intratumoral hemorrhage. 736 26

Five brain tumors in infancy were studied histopathologically. Though four of them could be included in primitive neuroectodermal tumor, histopathological findings were diverse. The diagnoses were medulloblastoma, cerebellar neuroblastoma, pineoblastoma, ependymoblastoma and ependymoma. While conventional stain for microscopy exhibited their specific findings, immunohistochemical and electron microscopical studies revealed morphological findings related functional structures of the tumor cells. These methodology is mandatory in order to elucidate cytogenesis and differentiation of infantile brain tumors.
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PMID:Pathology of infantile brain tumor. 769 30

In order to elucidate the role of inflammatory cytokines in the central nervous system, we examined the production of two leukocyte chemoattractants, IL-8 and monocyte chemotactic and activating factor (MCAF) in brain tumor cell lines. The glioma cell lines tested exhibited high levels of IL-8 and MCAF mRNA expression upon stimulation with IL-1 or TNF-alpha, while none of the neuroblastoma cell lines expressed these cytokine mRNA. Both IL-8 and MCAF mRNA expression depended on the dose of IL-1 alpha and TNF-alpha and appeared very rapidly, reaching maximal levels at 3-6 hr, with substantial production of these cytokines in the culture supernatants. When various immunosuppressive drugs were tested, glucocorticoids but not other immunosuppressive drugs markedly inhibited the IL-1 or TNF-alpha-induced IL-8 and MCAF mRNA accumulation, suggesting that glucocorticoid is a potent regulator of these inflammatory cytokine production in the neural tissues. In addition, reverse transcription-polymerase chain reaction (RT-PCR) revealed the expression of IL-8 and MCAF mRNA expression in resected brain tumor tissues including glioblastoma, astrocytoma grade 2, ependymoma and medulloblastoma, indicating that these inflammatory cytokines are expressed in vivo.
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PMID:Induction and regulation of IL-8 and MCAF production in human brain tumor cell lines and brain tumor tissues. 811 36

The frequency of different malignant cutaneous tumors (MCTs), primary and metastatic, in children is not known. We reviewed all MCTs, primary and metastatic, seen during a 20-year period in a large general pediatric hospital. Fifty-three MCTs, 36 primary and 17 metastatic, were diagnosed in 36,207 pediatric dermatology patients. The incidence was 1.4 per 1000 patients. The relative frequency of occurrence of the different tumors was as follows: rhabdomyosarcoma, 25%; lymphomas, 19%; basal cell carcinoma, 13%; leukemia, 13%; neuroblastoma, 10%; malignant melanoma, 6%; squamous cell carcinoma, 6%; unclassified sarcomas, 4%; epithelioid schwannoma, 2%; ependymoma, 2%. The mean follow-up was 3 years; 48% died, 27% were lost to follow-up, and 25% are under control. We conclude that primary and metastatic MCTs in children are rare. Their types differ from MCTs in an older age population. MCTs in children are associated with a high mortality rate, often related to late recognition.
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PMID:Malignant cutaneous tumors in children. Twenty years of experience at a large pediatric hospital. 828 84

Twenty-five primary ovarian neuroectodermal tumors occurred in females from 6 to 69 (average, 23) years of age; they had the usual presenting symptoms of abdominal swelling or pain. The tumors, which varied from cystic to solid, ranged from 4 to 20 cm (average, 14 cm) in diameter. Microscopic examination revealed three histologic categories--differentiated, primitive, and anaplastic--with the tumors in the first group having a better prognosis than those in the other two groups. Five of the six differentiated gliomas were pure ependymomas, and one was an ependymoma with an astrocytoma component; none contained teratomatous elements. Two patients with stage I tumors were alive 4 and 5 years postoperatively. The one patient with stage IIA tumor was free of disease at 3 years; one of the two patients with a stage III tumor died of tumor after 5 years, and one had two recurrences but was alive and well at 5 years. Twelve tumors were primitive, resembling medulloepithelioma, ependymoblastoma, neuroblastoma or medulloblastoma. Seven tumors had teratomatous foci of other types, including three dermoid cysts. Three patients with stage I tumors were alive at 7 months, 3 years, and 9 years postoperatively; six of seven patients with stage III tumors died of tumor 2 to 20 months postoperatively, and one was alive with disease at 1 year. Seven tumors were anaplastic, resembling glioblastoma. All contained foci of squamous epithelium. One patient with stage IA tumor died of tumor at 2 years, but two were free of tumor after 3 and 4 years. One patient with a stage IIA tumor died of disease after 5 years; another was alive with tumor at 1 year. One patient with a stage III tumor died after 4 months. The differential diagnosis of neuroectodermal tumors of the ovary includes many primary and metastatic ovarian neoplasms of diverse types, and distinction among them is important. Neuroectodermal tumors should be considered when examining unusual ovarian tumors, particularly if the patient is young.
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PMID:Primary neuroectodermal tumors of the ovary. A report of 25 cases. 839 2

Cells with uniform, small-round nucleus and clear cytoplasm (oligodendroglial-like cell, OLC) are commonly observed in central nervous system (CNS) neoplasm of glial and neuronal lineage, such as oligodendroglioma, clear-cell ependymoma, and central neurocytoma. Immunohistochemistry does not always contribute to the characterization of OLC because of (1) loss of antigen expression; (2) lack of specific markers for oligodendrogliomas; and (3) occasional coexpression of neuronal and glial antigens. An ultrastructural analysis associated with an immunohistochemical study of 20 cases of CNS tumors largely constituted by OLCs has been performed. Neurocytomas (12 cases), medullocytomas (2 cases), cerebral neuroblastoma (1 case), and ganglioglioma (1 case) showed OLCs with ultrastructural features of neuronal differentiation (neuritic processes, dense-core granules, synaptic structures). Oligodendroglioma (3 cases) OLCs were characterized by mitochondrial-rich cytoplasm, and ependymoma (1 case) OLCs showed microrosettes and scattered cilia. The electron microscopic analysis can provide a more precise diagnosis of these OLC-containing tumors despite their uniform morphological appearance.
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PMID:Ultrastructural characterization of oligodendroglial-like cells in central nervous system tumors. 894 Jul 61

The localization of cystatin C (CC) and transthyretin (TTR) synthesis was studied using Northern blot and immunohistochemical methods. Normal brain tissues from all sites studied contained CC mRNA. Immunoreactive CC was present in the choroid plexus epithelial cells, cerebral and cerebellar neurons, astrocytes, ependymal cells, macrophage-like cells of the arachnoid membrane and in neuroendocrine cells of the anterior pituitary lobe. TTR mRNA and TTR were restricted to the choroid plexus. In primary brain tumors, the transcript for CC was found in all 39 tumors examined, while the protein could only be demonstrated in 3/5 choroid plexus papillomas, 8/8 astrocytomas, 7/23 anaplastic astrocytomas and glioblastomas, 1/6 oligodendrogliomas, 1/1 oligoastrocytoma, 1/4 anaplastic oligodendrogliomas, 3/7 ependymomas, 0/1 anaplastic ependymoma, 0/5 primitive neuroectodermal tumors, 0/1 neuroblastoma, 3/11 meningiomas and 16/16 pituitary adenomas. CC cannot be used as a marker for any specific brain tumor type but the fact that the protein could be demonstrated more frequently in astrocytomas than in their more malignant counterparts suggests that the cellular production and secretion of CC changes with the malignant progression of these tumors. TTR mRNA and TTR were present only in the choroid plexus papillomas, indicating that TTR synthesis is mainly restricted to such brain neoplasms.
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PMID:Cystatin C and transthyretin expression in normal and neoplastic tissues of the human brain and pituitary. 914 88


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