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)

Using the avidin-biotin complex immunoperoxidase technique and antibodies to myoglobin, desmin, CLA, NSE, GFAP, keratin, fibronectin, alpha 1AT, lysozyme, S-100 protein, vimentin, cytokeratin, actin, the authors studied 60 cases of rhabdomyosarcoma (RMS) histopathologically diagnosed previously. Thirty-six cases showed both myoglobin and desmin positive stain, an objective evidence of the origin from skeletal muscles. The other 24 cases were identified as of non-skeletal muscle origin, including MFH, lymphoma, melanoma, neuroblastoma, malignant neurilemmoma, leiomyosarcoma etc. This study strongly suggests that histologic examination of RMS may lead to incorrect diagnosis. Histologically MFH and other types of spindle cell sarcomas invading normal skeletal muscles may be confused with pleomorphic RMS, lymphoma and neuroblastoma may be confused with embryonic RMS. Our findings indicate that myoglobin is a highly sensitive and specific tumor marker for RMS.
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PMID:[Immunohistochemical differential diagnosis of 60 cases of rhabdomyosarcoma]. 166 97

There is evidence that the gene for gamma-gamma enolase (neuron specific enolase, NSE) is regulated during cell differentiation and development, conserved in a variety of organisms and contains mRNA destabilizing sequences. In order to investigate further the mechanisms of these processes and to obtain large quantity of this protein, the NSE gene was isolated from neuroblastoma cells and cloned in E. coli using standard molecular biology techniques. The NSE gene expression was studied and the expressed protein (recombinant NSE) was characterized extensively. The recombinant NSE behaves like parental NSE in antisera specificity, resistance for chaotropic agents like urea, thermal stability at higher temperatures etc. The physical parameters like secondary structure, hydrophilicity, antigenic index and flexibility of the expressed protein were studied. The results of the present investigation collectively form the basis for initial investigations of how the expression of NSE gene is regulated. This is the first report where the recombinant NSE gene has been characterized so extensively.
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PMID:Expression of DNA sequences containing neuron specific enolase gene in Escherichia coli. 170 32

In this report the morphological and immunohistochemical features with respect to intermediate filament proteins and the neuroendocrine nature of bilateral neuroblastomas possibly originating from the adrenal medulla in a goat kid are presented. Histologically, the tumours were composed of small, round, blue cells organized in highly cellular nests and sheets separated by fibrovascular septa. Isolated cells mimicking the morphological features of neurones were observed in both tumours. Ultrastructurally, dense-core neurosecretory granules, about 100 nm in diameter, and irregularly organized neurotubular networks were seen. The small tumour cells were only labelled by vimentin, while the neurone-like cells were labelled by both neurone-specific enolase and synaptophysin. The lack of staining of the tumour cells by the neurofilament proteins antiserum, which was also observed in a human neuroblastoma (used as positive control) has been previously reported and is probably the result of the tissue processing and/or the poor differentiation stage of the tumours. The vimentin labelling of tumour cells could be explained by the poor differentiation stage of the tumours, since vimentin is the only intermediate filament protein in presumptive neuroblasts, being replaced by neurofilament proteins in later stages of neurogenesis.
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PMID:Caprine peripheral neuroblastomas: structural and ultrastructural features, intermediate filaments profile and neuro-endocrine characterization. 177 39

The prognosis of advanced neuroblastoma is extremely poor. We treated 5 patients with advanced neuroblastoma, older than 3 years, with multimodal therapy including intraoperative irradiation and autologous bone marrow transplantation. Elevated serum NSE and ferritin level and unfavorable histology according to the Shimadas histological classification, all of which are indicators of poor prognosis, were found in all of them. N-myc oncogene was amplified in 3 cases. After preoperative intensive induction chemotherapy, delayed primary operation and intraoperative irradiation (10-15 Gy) were performed. The postoperative lethal dose chemotherapy and total body irradiation (33 Gy x 3 days) were followed by autologous bone marrow transplantation. Tumor cells were purged using immunomagnetic beads method. Two cases showed recurrence (brain; 1, bone and bone marrow; 1) and a metastatic brain tumor was extirpated completely. All of them are alive during the follow up period from 6mo. to 4y8mo. (mean; 2y5mo.) with no evidence of disease except one. It may be concluded that our multimodal therapy is effective in achieving better results for advanced neuroblastoma.
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PMID:[Experience of multimodal therapy for advanced neuroblastoma]. 194 78

Nasal olfactory neuroblastoma and other malignant neoplasms showing spherical cell are easily confused in clinical and pathological diagnosis. We have made immunohistochemical staining in 7 olfactory neuroblastoma cases. Results showed that they were all positively stained by NSE, and negatively by keratin, leucocyte common antigen, desmin and S-100 protein (except 1 positive by desmin). We believe that the olfactory neuroblastoma cells are uniform morphologically. The cells are round, elliptic, having scant cytoplasm; with coarse or fine chromatin. Neurofibers can be found among tumor cells. Their histological structures vary greatly. They may form nest or garland, and they may also form striation, cleft or discrete structure; but rosette or pseudo-rosette structure is uncommon.
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PMID:[Clinicopathological features and immunohistological diagnosis of olfactory neuroblastoma]. 225 15

In 1982, Hassoun et al. reported two cases of differentiated neuroblastoma with the clinical and light microscopic appearance of intraventricular oligodendroglioma and gave a name of "central neurocytoma" to this tumor. Jerdan et al. (1983) called the similar tumor as "differentiated cerebral neuroblastoma in adults". As the tumor can be diagnosed only by ultrastructural study, the established cases so far reported are very rare. In this paper we present five cases identical to those presented by Hassoun et al. and clarify the essential nature of this new category of brain tumors. All of our cases of central neurocytoma occurred in the lateral ventricles of young adults. Clinically there was no evidence of leptomeningeal or ventricular dissemination of tumor cells. After subtotal resection of the tumor and 6000 rads of whole brain irradiation, the tumor mass disappeared and no evidence for recurrence of the tumor was noted on CT scan. All cases showed almost the same histology. The tumor cells contained a small round and/or oval nucleus, and had eosinophilic thin delicate cytoplasmic processes. There were no Homer Wright rosette, but were anuclear spaces consisting of fine fibrillar structures, like so called "broad rosettes" or "large rosettes". Capillary mesh was found among the tumor cells, but there was no endothelial proliferation. The tumor cells were monotonous, lacking pleomorphism, mitotic figures, and necrotic foci. Calcifications were observed in two cases. In the areas where the tumor cells arranged loosely, cytoplasms became clear, showing perinuclear halo, like those of oligodendrogliomas. Immunohistochemical examination showed GFAP and vimentin positive cells were all reactive astrocytes around capillaries and near calcifications. No tumor cells contained GFAP and vimentin. The tumor cells were also negative for neurofilament both of 70 KD and 200 KD. NSE was more or less positive for tumor cell cytoplasm as well as fine fibrils. Anti-Leu7 antibodies stained only fine cytoplasmic processes, but not cytoplasm. Some reactive astrocytes were stained with anti-Leu7 antibodies. Electron microscopic examination showed nuclei of the tumor cells were roughly round or oval without nuclear indentations and contained finely dispersed nuclear chromatin. In the cytoplasm, there were numerous free ribosomes, mitochondria, Golgi apparatus and electron dense various-shaped granules. Microtubules were found in the periphery of the cells, but filamentous structures were not identified.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:[A clinicopathological study of central neurocytoma]. 247 77

Esthesioneuroblastoma (ESTH) is a neuroepithelial-cell-derived neoplasm of the olfactory mucosa composed of homogeneous small round cells which contain neurosecretory granules. Melanin has been detected in such tumours only occasionally. Here we describe a new case of ESTH with divergent differentiation. The primary neoplasm was found in a 67 year-old female, involving the left nasal and maxillary sinus; she died of cerebral metastasis ten months after diagnosis. Histologically only small round cells were seen, with S-100 and NSE positivity. Electron microscopy revealed neurosecretory granules and filaments, as well as the occasional presence of melanosomes. A nude mice xenograft line has been established, and is presently in its ninth transfer. Two cell types are present: small round-to-spindle shaped cells with neural features, and large epithelial-like ones. Both immunohistochemistry and electron microscopy confirm this dual differentiation, with the presence of membrane-bound dense-core neural secretion, as well as melanosomes of neuroectodermal origin. Additionally, an in vitro cell line has been established. Cytogenetic analysis confirmed the presence of both malignant human melanoma patterns; non-random abnormalities in chromosomes 1 and 6, extra copies of chromosome 7. Duplication of the long arm of chromosome 14, as seen in olfactory neuroblastoma, is also seen.
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PMID:Pigmented esthesioneuroblastoma showing dual differentiation following transplantation in nude mice. An immunohistochemical, electron microscopical, and cytogenetic analysis. 249

A small round cell tumor of soft tissue arising in the retroperitoneum of an 18-year-old woman is reported. The light and electron microscopic features of the tumor were mostly indistinguishable from Ewing's sarcoma of the bone and in some parts from differentiating neuroblastoma with mature neurons and Schwann cells. An immunohistochemical study using anti-NSE antibody showed many widespread NSE-positive cells lying singly and, more often, in clusters including the undifferentiated areas. The tumor was not anatomically related to the paravertebral sympathetic ganglia or adrenal glands. The present findings strongly suggest that certain extraskeletal Ewing's sarcomas are very primitive neuroectodermal tumors.
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PMID:Neuroectodermal differentiation in "extraskeletal Ewing's sarcoma". 256 Mar 12

The ultrastructural appearances of 7 primitive neuroepithelial tumours (PNETs) originating in soft tissues and bone are described. Three of the tumours represented primary soft tissue lesions, while locally recurrent tumour or pulmonary metastases were studied from the 4 skeletal tumours, all of which had been diagnosed previously as Ewing's sarcomas. Rosettes were present in one of the soft tissue lesions and although not seen in the primary skeletal neoplasms, they were identified by light microscopy (LM) in 2 of 3 pulmonary metastases, one of which had the morphology of a neuroepithelioma, with innumerable Homer Wright rosettes. Conventional TEM revealed cytoplasmic processes in all cases and rosettes in varying stages of development were also evident, but the appearances did not achieve the level of cellular organization seen in neuroblastoma: microtubules were few, while dense-core granules varied in number but were generally sparse and pleomorphic, resembling lysosomes. However, typical neurosecretory granules were found in one lung metastasis; the neoplastic cells comprising the same tumour also had epithelial markers in the form of well constructed desmosomes, while freeze-fracture analysis demonstrated elaborate tight junctions. In thin sections, junctions in the other tumours appeared rudimentary, but freeze-fracture of a further case revealed small collections of membrane particles suggesting extremely poorly developed desmosomes. Immunocytochemical study of 4 tumours (2 originating in soft tissue and 2 in bone) demonstrated weak to moderate immunostaining for neurone-specific enolase and with several monoclonal antibodies reactive with neuroblastomas, but there was no evidence of immunolabelling for tyrosine hydroxylase.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Primitive neuroepithelial tumours of soft tissues and of bone: further ultrastructural and immunocytochemical clarification of 'Ewing's sarcoma', including freeze-fracture analysis. 264 32

Despite the fact that olfactory neuroblastoma is considered to be a rare malignant neoplasm of the sinonasal cavity, its frequent inclusion in the differential diagnosis of small-cell neoplasms of the nasal cavity is commonplace. Differential diagnosis of small round tumors solely on the basis of a light-microscopic examination of a hematoxylin and eosin stain often can be impossible. However, immunohistochemical stains such as NSE, S-100, and chromogranin immunostain can be very helpful in diagnosing olfactory neuroblastoma. If special stains are noncontributory, the diagnosis may hinge on supporting evidence from a series of electron micrographs of a properly selected and prepared tumor.
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PMID:Olfactory neuroblastoma. 266 27


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