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KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Abbreviations
Term primitive neuroectodermal tumor (PNET) no longer used!
2016 WHO classification of embryonal tumors has 3 major groups
Medulloblastoma (MB)
Embryonal tumor with multilayered rosettes, C19MC altered (ETMR)
Atypical teratoid/rhabdoid tumor (AT/RT)
Definitions
ETMRs have both primitive embryonal regions, neoplastic neuropil
ETMR includes neoplasms previously termed
Primitive neuroepithelial tumor (PNET)
Embryonal tumor with abundant neuropil and true rosettes (ETANTR)
Ependymoblastoma
Medulloepithelioma
ETMR, not otherwise specified (NOS)
Multilayered ependymoblastic rosettes identified
No C19MC alteration
IMAGING
General Features
CT Findings
MR Findings
Ultrasonographic Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
Li BK et al: Pediatric embryonal brain tumors in the molecular era. Expert Rev Mol Diagn. 20(3):293-303, 2020
de Kock L et al: An update on the central nervous system manifestations of DICER1 syndrome. Acta Neuropathol. 139(4):689-701, 2019
Lambo S et al: The molecular landscape of ETMR at diagnosis and relapse. Nature. 576(7786):274-80, 2019
Pei YC et al: Embryonal tumor with multilayered rosettes, C19MC-altered (ETMR): a newly defined pediatric brain tumor. Int J Clin Exp Pathol. 12(8):3156-63, 2019
Uro-Coste E et al: ETMR-like infantile cerebellar embryonal tumors in the extended morphologic spectrum of DICER1-related tumors. Acta Neuropathol. 137(1):175-7, 2019
Bouali S et al: Embryonal tumor with multilayered rosettes: illustrative case and review of the literature. Childs Nerv Syst. 34(12):2361-9, 2018
Shah AH et al: Extracranial extra-CNS spread of embryonal tumor with multilayered rosettes (ETMR): case series and systematic review. Childs Nerv Syst. 34(4):649-54, 2018
Sin-Chan P et al: Molecular classification and management of rare pediatric embryonal brain tumors. Curr Oncol Rep. 20(9):69, 2018
Wang B et al: Embryonal tumor with multilayered rosettes, C19MC-altered: clinical, pathological, and neuroimaging findings. J Neuroimaging. 28(5):483-9, 2018
Korshunov A et al: Embryonal tumor with abundant neuropil and true rosettes (ETANTR), ependymoblastoma, and medulloepithelioma share molecular similarity and comprise a single clinicopathological entity. Acta Neuropathol. 128(2):279-89, 2014
Spence T et al: CNS-PNETs with C19MC amplification and/or LIN28 expression comprise a distinct histogenetic diagnostic and therapeutic entity. Acta Neuropathol. 128(2):291-303, 2014
Related Anatomy
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Related Differential Diagnoses
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Abbreviations
Term primitive neuroectodermal tumor (PNET) no longer used!
2016 WHO classification of embryonal tumors has 3 major groups
Medulloblastoma (MB)
Embryonal tumor with multilayered rosettes, C19MC altered (ETMR)
Atypical teratoid/rhabdoid tumor (AT/RT)
Definitions
ETMRs have both primitive embryonal regions, neoplastic neuropil
ETMR includes neoplasms previously termed
Primitive neuroepithelial tumor (PNET)
Embryonal tumor with abundant neuropil and true rosettes (ETANTR)
Ependymoblastoma
Medulloepithelioma
ETMR, not otherwise specified (NOS)
Multilayered ependymoblastic rosettes identified
No C19MC alteration
IMAGING
General Features
CT Findings
MR Findings
Ultrasonographic Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
Li BK et al: Pediatric embryonal brain tumors in the molecular era. Expert Rev Mol Diagn. 20(3):293-303, 2020
de Kock L et al: An update on the central nervous system manifestations of DICER1 syndrome. Acta Neuropathol. 139(4):689-701, 2019
Lambo S et al: The molecular landscape of ETMR at diagnosis and relapse. Nature. 576(7786):274-80, 2019
Pei YC et al: Embryonal tumor with multilayered rosettes, C19MC-altered (ETMR): a newly defined pediatric brain tumor. Int J Clin Exp Pathol. 12(8):3156-63, 2019
Uro-Coste E et al: ETMR-like infantile cerebellar embryonal tumors in the extended morphologic spectrum of DICER1-related tumors. Acta Neuropathol. 137(1):175-7, 2019
Bouali S et al: Embryonal tumor with multilayered rosettes: illustrative case and review of the literature. Childs Nerv Syst. 34(12):2361-9, 2018
Shah AH et al: Extracranial extra-CNS spread of embryonal tumor with multilayered rosettes (ETMR): case series and systematic review. Childs Nerv Syst. 34(4):649-54, 2018
Sin-Chan P et al: Molecular classification and management of rare pediatric embryonal brain tumors. Curr Oncol Rep. 20(9):69, 2018
Wang B et al: Embryonal tumor with multilayered rosettes, C19MC-altered: clinical, pathological, and neuroimaging findings. J Neuroimaging. 28(5):483-9, 2018
Korshunov A et al: Embryonal tumor with abundant neuropil and true rosettes (ETANTR), ependymoblastoma, and medulloepithelioma share molecular similarity and comprise a single clinicopathological entity. Acta Neuropathol. 128(2):279-89, 2014
Spence T et al: CNS-PNETs with C19MC amplification and/or LIN28 expression comprise a distinct histogenetic diagnostic and therapeutic entity. Acta Neuropathol. 128(2):291-303, 2014
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