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)

Seven cases of primary cerebral neuroectodermal tumors with predominant neuroblastic features were studied ultrastructurally and five were evaluated immunohistochemically. The fine structural features were indicative of neuroblastic differentiation by the presence of elongated cytoplasmic processes, electron-dense neurosecretory granules, and neurotubules. Five of the seven cases had the morphologic findings of classic cerebral neuroblastoma, and the sixth case, originally diagnosed as an oligodendroglioma, had the features of a differentiated neuroblastoma. Desmoplastic and/or stromal foci were intermingled with neuronal-ganglionic cells and neuroblasts in the seventh case. In addition to strong immunoreactivity for S-100 protein and glial fibrillary acidic protein in the desmoplastic areas, the spindle cells had fibroblastic and Schwannian features by electron microscopy in the latter case. The neuroblastic cells and fibrillary network were immunoreactive for neuron-specific enolase and neurofilament in the five study cases. It is concluded that cerebral neuroectodermal tumors may express an range of phenotypic features from the exclusive neuroblastic stage to a neuronal and stromogenic phase analogous to the classic neuroblastoma of the sympathetic nervous system.
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PMID:Primary cerebral neuroectodermal tumors: neuroblastoma, differentiated neuroblastoma, and composite neuroectodermal tumor. 319 93

In the publication by the World Health Organization of the histological classification of central nervous system tumors in 1993, several new tumor types were added, including pleomorphic xanthoastrocytoma (PXA), dysembryo-plastic neuroepithelial tumor (DNT), and desmoplastic infantile ganglioglioma (DIG). Referring to these newly classified tumors of the brain, the present article describes the neuroradiological findings of relatively rare tumors of the brain such as PXA, subependymoma, gangliocytoma, dysplastic cerebellar gangliocytoma (Lhermitte-Duclos disease), DIG, central neurocytoma (CN), neuroblastoma, desmoplastic medulloblastoma, atypical teratoid/rhabdoid tumor (ATRT), glossopharyngeal schwannoma, and malignant fibrous histiocytoma (MFH). PXA and DIG affect the cerebral hemisphere and appear to be cystic masses with a solid component on CT and MRI. Gangliocytomas commonly occur in the floor of the third ventricle and the temporal lobe. CT and MRI typically show a mass lesion with no vasogenic edema. In Lhermitte-Duclos disease, T2-weighted MR images reveal characteristic enlarged folia. Desmoplastic medulloblastomas tend to favor the cerebral hemisphere in the adolescent. ATRTs are rarely seen in infants or children and resemble medulloblastomas or primitive neuroectodermal tumors. Intracranial MFHs are also rare tumors and sometimes resemble the meningiomas.
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PMID:[Neuroradiological findings of relatively rare tumors of the brain]. 1223 62

The diagnosis of pediatric tumors relies heavily on immunohistochemical staining of small tissue biopsies, since many entities share a "small blue cell" phenotype. More recently, molecular genetic analysis for detection of specific gene fusion products has become available. With the increased use of such molecular techniques, the authors have noted that tumors with proven molecular diagnoses can exhibit unusual patterns of immunohistochemical staining. This study examines pediatric tumors with a "small blue cell" phenotype in which molecular diagnoses were available where applicable. A panel of immunohistochemical stains was performed (S100, CD56, NB84, CD99 [MIC2], Bcl-2, CD117, CD34, desmin, MNF116, and WT1). In the 370 sections from 37 cases, all primitive neuroectodermal tumors, with and without the presence of t(11;22), demonstrated uniform membranous membrane staining with CD99 (MIC2) and focal staining with CD56, NB84, MNF116, and WT1. All rhabdomyosarcomas, both alveolar and embryonal, demonstrated uniform desmin, CD56, and cytoplasmic WT1 immunostaining. Desmoplastic small round cell tumors showed positive cytokeratin staining, with half having "dot-like" cytoplasmic desmin and WT1 positivity; some showed focal positivity for NB84, CD99, and Bcl-2. The "undifferentiated" sarcomas showed the widest range of staining, with no marker staining all cases. Neuroblastomas exhibited uniform strong staining for CD56 and NB84 and marked cytoplasmic Bcl-2 positivity, and some cases showed cytoplasmic WT1 expression. Blastematous Wilms' tumors showed uniform strong membranous staining for CD56, uniform cytoplasmic staining for Bcl-2, and nuclear expression of WT1. Embryonal pediatric malignancies can demonstrate apparently nonspecific expression patterns for several antigens, which may reflect developmental immaturity rather than specific differentiation pathways.
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PMID:Immunohistochemical findings in embryonal small round cell tumors with molecular diagnostic confirmation. 1572 86