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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.

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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)
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  • C19MC amplification (en)
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  • Embryonal tumor with multilayered rosettes (en)
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  • 6.31152E7
dbp:prognosis
  • 2.6298E7
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  • Until recently, recognized as medulloepithelioma, ependymoblastoma, and embryonal tumor with abundant neuropil and true rosettes (en)
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  • 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)
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  • Embryonal tumor with multilayered rosettes (ETMR) (en)
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