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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
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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
