Embryonal tumour with multilayered rosettes (original) (raw)
Der Embryonale Tumor mit mehrreihigen Rosetten (ETMR), auch Embryonaler Tumor mit mehrschichtigen Rosetten genannt, ist ein sehr seltener kindlicher Hirntumor und wird zu den embryonalen Tumoren des ZNS gezählt und nach der WHO-Klassifikation der Tumoren des zentralen Nervensystems dem WHO Grad IV zugeordnet. Der sehr seltene früher als Medulloepitheliom bezeichnete bösartige Hirntumor wird jetzt als ETMR eingeordnet, ebenso das Ependymoblastom. Dieser Tumor ist nicht zu verwechseln mit dem Rosettenbildenden glioneuronalen Tumor.
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| dbo:abstract | Der Embryonale Tumor mit mehrreihigen Rosetten (ETMR), auch Embryonaler Tumor mit mehrschichtigen Rosetten genannt, ist ein sehr seltener kindlicher Hirntumor und wird zu den embryonalen Tumoren des ZNS gezählt und nach der WHO-Klassifikation der Tumoren des zentralen Nervensystems dem WHO Grad IV zugeordnet. Der sehr seltene früher als Medulloepitheliom bezeichnete bösartige Hirntumor wird jetzt als ETMR eingeordnet, ebenso das Ependymoblastom. Dieser Tumor ist nicht zu verwechseln mit dem Rosettenbildenden glioneuronalen Tumor. (de) Embryonal tumor with multilayered rosettes (ETMR) is an embryonal central nervous system tumor. It is considered an embryonal tumor because it arises from cells partially differentiated or still undifferentiated from birth, usually neuroepithelial cells, stem cells destined to turn into glia or neurons. It can occur anywhere within the brain and can have multiple sites of origins, with a high probability of metastasis through cerebrospinal fluid (CSF). Metastases outside the central nervous system have been reported, but remain rare. Until recently, ETMRs were not recognized as a standalone entity and were grouped together with other CNS primitive neuroectodermal tumors. Histologically, it is similar to other CNS embryonal tumors, such as medulloblastoma, but different regarding genetic factors. It is a rare disease occurring in less than 1 in 700,000 children under the age of 4. Symptoms depend on the location of the tumor and, thus, may vary, but they may include raised intracranial pressure, paresis, seizures, visual impairments, ataxia, and torticollis. A biopsy is needed to establish diagnosis, which is done by molecular analysis of the sample. A standard treatment plan hasn't been established; common strategies involve chemotherapy and radiotherapy for individuals older than 3 years of age. Their efficacy, however, is still controversial. Surgery can be used to remove the tumor, but it often recurs. (en) |
| dbo:medicalDiagnosis | dbr:CT_scan dbr:Tissue_biopsy dbr:MRI_scan |
| dbo:treatment | dbr:Surgery dbr:Chemotherapy dbr:Radiation_therapy |
| dbo:wikiPageExternalLink | https://web.archive.org/web/20080513082649/http:/www.ninds.nih.gov/disorders/brainandspinaltumors/detail_brainandspinaltumors.htm |
| dbo:wikiPageID | 69340610 (xsd:integer) |
| dbo:wikiPageLength | 7384 (xsd:nonNegativeInteger) |
| dbo:wikiPageRevisionID | 1103533427 (xsd:integer) |
| dbo:wikiPageWikiLink | dbr:LIN28A_(gene) dbr:Medulloblastoma dbr:Medulloepithelioma dbr:Neuron dbr:Life_expectancy dbr:Surgery dbr:CT_scan dbc:Pediatric_cancers dbr:Central_nervous_system_primitive_neuroectodermal_tumor dbr:Central_nervous_system_tumor dbc:Small-blue-round-cell_tumors dbr:Dicer dbr:Glia dbr:Ependymoblastoma dbc:Brain_tumor dbr:Chemotherapy dbr:C19MC_miRNA_cluster dbr:Metastasis dbr:Neuro-Oncology dbr:Neurosurgery dbr:Radiation_therapy dbr:Cerebrospinal_fluid dbr:Neuroepithelial_cell dbr:WHO_classification_of_tumours_of_the_central_nervous_system dbr:Tissue_biopsy dbr:MRI_scan |
| dbp:causes | C19MC amplification (en) |
| dbp:diagnosis | dbr:CT_scan dbr:Tissue_biopsy dbr:MRI_scan |
| dbp:field | dbr:Neuro-Oncology dbr:Neurosurgery |
| dbp:name | Embryonal tumor with multilayered rosettes (en) |
| dbp:onset | 6.31152E7 |
| dbp:prognosis | 2.6298E7 |
| dbp:synonyms | Until recently, recognized as medulloepithelioma, ependymoblastoma, and embryonal tumor with abundant neuropil and true rosettes (en) |
| dbp:treatment | dbr:Surgery dbr:Chemotherapy dbr:Radiation_therapy |
| dbp:wikiPageUsesTemplate | dbt:Infobox_medical_condition_(new) dbt:Short_description dbt:Nervous_tissue_tumors |
| dcterms:subject | dbc:Pediatric_cancers dbc:Small-blue-round-cell_tumors dbc:Brain_tumor |
| rdf:type | owl:Thing wikidata:Q12136 dbo:Disease |
| rdfs:comment | Der Embryonale Tumor mit mehrreihigen Rosetten (ETMR), auch Embryonaler Tumor mit mehrschichtigen Rosetten genannt, ist ein sehr seltener kindlicher Hirntumor und wird zu den embryonalen Tumoren des ZNS gezählt und nach der WHO-Klassifikation der Tumoren des zentralen Nervensystems dem WHO Grad IV zugeordnet. Der sehr seltene früher als Medulloepitheliom bezeichnete bösartige Hirntumor wird jetzt als ETMR eingeordnet, ebenso das Ependymoblastom. Dieser Tumor ist nicht zu verwechseln mit dem Rosettenbildenden glioneuronalen Tumor. (de) Embryonal tumor with multilayered rosettes (ETMR) is an embryonal central nervous system tumor. It is considered an embryonal tumor because it arises from cells partially differentiated or still undifferentiated from birth, usually neuroepithelial cells, stem cells destined to turn into glia or neurons. It can occur anywhere within the brain and can have multiple sites of origins, with a high probability of metastasis through cerebrospinal fluid (CSF). Metastases outside the central nervous system have been reported, but remain rare. (en) |
| rdfs:label | Embryonaler Tumor mit mehrreihigen Rosetten (de) Embryonal tumour with multilayered rosettes (en) |
| owl:sameAs | wikidata:Embryonal tumour with multilayered rosettes dbpedia-de:Embryonal tumour with multilayered rosettes https://global.dbpedia.org/id/FuLDn |
| prov:wasDerivedFrom | wikipedia-en:Embryonal_tumour_with_multilayered_rosettes?oldid=1103533427&ns=0 |
| foaf:isPrimaryTopicOf | wikipedia-en:Embryonal_tumour_with_multilayered_rosettes |
| foaf:name | Embryonal tumor with multilayered rosettes (ETMR) (en) |
| is dbo:wikiPageWikiLink of | dbr:WHO_classification_of_tumours_of_the_central_nervous_system |
| is foaf:primaryTopic of | wikipedia-en:Embryonal_tumour_with_multilayered_rosettes |