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Article: Dysembryoplastic neuroepithelial tumor: A tumor with small neuronal cells resembling oligodendroglioma

TitleDysembryoplastic neuroepithelial tumor: A tumor with small neuronal cells resembling oligodendroglioma
Authors
Issue Date1994
PublisherLippincott Williams & Wilkins. The Journal's web site is located at http://www.ajsp.com
Citation
American Journal Of Surgical Pathology, 1994, v. 18 n. 6, p. 604-614 How to Cite?
AbstractDysembryoplastic neuroepithelial tumor (DNT) is a clinicopathologically unique group of tumors, mostly located in the temporal lobe, associated with intractable complex partial seizure in young patients. We report two unusual cases with multifocal involvement of diverse sites in the central nervous system. Case 1 is that of a 50-year-old man with 9-year history of grand mal seizures, who died of acute myocardial infarction. Case 2 is that of a 10- year-old girl with intractable complex partial seizures and behavioral disorder. Postmortem examination in case 1 showed multifocal tumor in the left temporal lobe, third ventricle, and basal ganglia. Magnetic resonance imaging in case 2 showed tumor in the right temporal lobe, both thalami, right cerebellar hemisphere, and pons. Histologically, both tumors were characterized by a multinodular appearance with a predominant component of alveolar arrangement of oligodendroglial-like cells around delicate capillaries, with mucoid matrix containing floating ganglion cells. There were also astrocytic nodules resembling pilocytic astrocytoma in case 1, and a gangliocytoma-like area merging with surrounding cortical dysplasia in case 2. Ultrastructural examination showed ganglionic differentiation in the oligodendroglial-like cells in case 2. They possessed dense core neurosecretory granules and many slender neuritic processes with microtubules arranged in parallel and terminating in synaptic junctions. The periventricularly located tumor with nodular extension to the periphery suggests an origin from subependymal germinal matrix with nests of primitive neuroblasts arrested in their embryonal migration. DNTs are related to ganglioglioma based on their common location and clinical behavior and on the presence of both ganglionic and astrocytic cells. They are also related to pilocytic astrocytoma by morphological and behavioral similarity. Together with cerebral neuroblastoma and central neurocytoma, they form a spectrum of tumors harboring small neuronal cells. The differentiation of DNT from oligodendroglioma is important so as to avoid unnecessarily aggressive therapy.
Persistent Identifierhttp://hdl.handle.net/10722/148025
ISSN
2015 Impact Factor: 4.951
2015 SCImago Journal Rankings: 2.393
ISI Accession Number ID

 

DC FieldValueLanguage
dc.contributor.authorLeung, SYen_US
dc.contributor.authorGwi, Een_US
dc.contributor.authorNg, HKen_US
dc.contributor.authorFung, CFen_US
dc.contributor.authorYam, KYen_US
dc.date.accessioned2012-05-29T06:10:27Z-
dc.date.available2012-05-29T06:10:27Z-
dc.date.issued1994en_US
dc.identifier.citationAmerican Journal Of Surgical Pathology, 1994, v. 18 n. 6, p. 604-614en_US
dc.identifier.issn0147-5185en_US
dc.identifier.urihttp://hdl.handle.net/10722/148025-
dc.description.abstractDysembryoplastic neuroepithelial tumor (DNT) is a clinicopathologically unique group of tumors, mostly located in the temporal lobe, associated with intractable complex partial seizure in young patients. We report two unusual cases with multifocal involvement of diverse sites in the central nervous system. Case 1 is that of a 50-year-old man with 9-year history of grand mal seizures, who died of acute myocardial infarction. Case 2 is that of a 10- year-old girl with intractable complex partial seizures and behavioral disorder. Postmortem examination in case 1 showed multifocal tumor in the left temporal lobe, third ventricle, and basal ganglia. Magnetic resonance imaging in case 2 showed tumor in the right temporal lobe, both thalami, right cerebellar hemisphere, and pons. Histologically, both tumors were characterized by a multinodular appearance with a predominant component of alveolar arrangement of oligodendroglial-like cells around delicate capillaries, with mucoid matrix containing floating ganglion cells. There were also astrocytic nodules resembling pilocytic astrocytoma in case 1, and a gangliocytoma-like area merging with surrounding cortical dysplasia in case 2. Ultrastructural examination showed ganglionic differentiation in the oligodendroglial-like cells in case 2. They possessed dense core neurosecretory granules and many slender neuritic processes with microtubules arranged in parallel and terminating in synaptic junctions. The periventricularly located tumor with nodular extension to the periphery suggests an origin from subependymal germinal matrix with nests of primitive neuroblasts arrested in their embryonal migration. DNTs are related to ganglioglioma based on their common location and clinical behavior and on the presence of both ganglionic and astrocytic cells. They are also related to pilocytic astrocytoma by morphological and behavioral similarity. Together with cerebral neuroblastoma and central neurocytoma, they form a spectrum of tumors harboring small neuronal cells. The differentiation of DNT from oligodendroglioma is important so as to avoid unnecessarily aggressive therapy.en_US
dc.languageengen_US
dc.publisherLippincott Williams & Wilkins. The Journal's web site is located at http://www.ajsp.comen_US
dc.relation.ispartofAmerican Journal of Surgical Pathologyen_US
dc.subject.meshBrain Neoplasms - Complications - Diagnosis - Pathologyen_US
dc.subject.meshCerebellum - Pathologyen_US
dc.subject.meshChilden_US
dc.subject.meshEpilepsy, Complex Partial - Complicationsen_US
dc.subject.meshFemaleen_US
dc.subject.meshHumansen_US
dc.subject.meshMagnetic Resonance Imagingen_US
dc.subject.meshMaleen_US
dc.subject.meshMiddle Ageden_US
dc.subject.meshNeurons - Pathologyen_US
dc.subject.meshOligodendroglioma - Pathologyen_US
dc.subject.meshSeizures - Complicationsen_US
dc.subject.meshTemporal Lobe - Pathologyen_US
dc.titleDysembryoplastic neuroepithelial tumor: A tumor with small neuronal cells resembling oligodendrogliomaen_US
dc.typeArticleen_US
dc.identifier.emailLeung, SY:suetyi@hkucc.hku.hken_US
dc.identifier.authorityLeung, SY=rp00359en_US
dc.description.naturelink_to_subscribed_fulltexten_US
dc.identifier.pmid8179075-
dc.identifier.scopuseid_2-s2.0-0028352108en_US
dc.identifier.volume18en_US
dc.identifier.issue6en_US
dc.identifier.spage604en_US
dc.identifier.epage614en_US
dc.identifier.isiWOS:A1994NL61500007-
dc.publisher.placeUnited Statesen_US

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