Includes former histologic diagnoses: Ependymal tumor with abundant neuropil & true rosettes (ETANTR) & ependymoblastoma
Definitions
Central nervous system primitive neuroectodermal tumor (CNS-PNET): Antiquated term for primitive cerebral embryonal tumor; no longer in WHO 2016
Umbrella term formerly containing CNS neuroblastoma, ependymoblastoma, medulloepithelioma, & ETANTR
Now molecularly & genetically distinct entities considered under embryonal tumors (all WHO grade IV) include
ETMR, C19MC altered
CNS embryonal tumor, not otherwise specified (NOS)
CNS Neuroblastoma
Medulloepithelioma
Medulloblastoma
Atypical teratoid/rhabdoid tumor
IMAGING
General Features
Radiographic Findings
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
Image Interpretation Pearls
Selected References
Dangouloff-Ros V et al: CT and multimodal MR imaging features of embryonal tumors with multilayered rosettes in children. AJNR Am J Neuroradiol. 40(4):732-6, 2019
Hwang EI et al: Extensive molecular and clinical heterogeneity in patients with histologically diagnosed CNS-PNET treated as a single entity: a report from the Children's Oncology Group randomized ACNS0332 trial. J Clin Oncol. JCO2017764720, 2018
Shih RY et al: Embryonal tumors of the central nervous system: from the radiologic pathology archives. Radiographics. 38(2):525-41, 2018
Wang B et al: Embryonal tumor with multilayered rosettes, C19MC-altered: clinical, pathological, and neuroimaging findings. J Neuroimaging. 28(5):483-9, 2018
Louis DN et al: The 2016 World Health Organization classification of tumors of the central nervous system: a summary. Acta Neuropathol. 131(6):803-20, 2016
Sturm D et al: New brain tumor entities emerge from molecular classification of CNS-PNETs. Cell. 164(5):1060-72, 2016
Biswas A et al: Treatment outcome and patterns of failure in patients of non-pineal supratentorial primitive neuroectodermal tumor: review of literature and clinical experience form a regional cancer center in north India. Acta Neurochir (Wien). 157(7):1251-66, 2015
Jakacki RI et al: Outcome and prognostic factors for children with supratentorial primitive neuroectodermal tumors treated with carboplatin during radiotherapy: a report from the Children's Oncology Group. Pediatr Blood Cancer. 62(5):776-83, 2015
Nowak J et al: Systematic comparison of MRI findings in pediatric ependymoblastoma with ependymoma and CNS primitive neuroectodermal tumor not otherwise specified. Neuro Oncol. 17(8):1157-65, 2015
Phillips J et al: Practical molecular pathology and histopathology of embryonal tumors. Surg Pathol Clin. 8(1):73-88, 2015
Lester RA et al: Clinical outcomes of children and adults with central nervous system primitive neuroectodermal tumor. J Neurooncol. 120(2):371-9, 2014
De Braganca KC et al: Treatment options for medulloblastoma and CNS primitive neuroectodermal tumor (PNET). Curr Treat Options Neurol. 15(5):593-606, 2013
Perreault S et al: Relapse patterns in pediatric embryonal central nervous system tumors. J Neurooncol. 115(2):209-15, 2013
von Bueren AO: CNS PNET molecular subgroups with distinct clinical features. Lancet Oncol. 13(8):753-4, 2012
Behdad A et al: Central nervous system primitive neuroectodermal tumors: a clinicopathologic and genetic study of 33 cases. Brain Pathol. 20(2):441-50, 2010
Pfister SM et al: Molecular diagnostics of CNS embryonal tumors. Acta Neuropathol. 120(5):553-66, 2010
Johnston DL et al: Supratentorial primitive neuroectodermal tumors: a Canadian pediatric brain tumor consortium report. J Neurooncol. 86(1):101-8, 2008
Chawla A et al: Paediatric PNET: pre-surgical MRI features. Clin Radiol. 62(1):43-52, 2007
Pfister S et al: Supratentorial primitive neuroectodermal tumors of the central nervous system frequently harbor deletions of the CDKN2A locus and other genomic aberrations distinct from medulloblastomas. Genes Chromosomes Cancer. 46(9):839-51, 2007
Inda MM et al: Genetic heterogeneity in supratentorial and infratentorial primitive neuroectodermal tumours of the central nervous system. Histopathology. 47(6):631-7, 2005
Pomeroy SL et al: Prediction of central nervous system embryonal tumour outcome based on gene expression. Nature. 415(6870):436-42, 2002
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
TERMINOLOGY
Abbreviations
Embryonal tumor with multilayered rosettes (ETMR)
Synonyms
Includes former histologic diagnoses: Ependymal tumor with abundant neuropil & true rosettes (ETANTR) & ependymoblastoma
Definitions
Central nervous system primitive neuroectodermal tumor (CNS-PNET): Antiquated term for primitive cerebral embryonal tumor; no longer in WHO 2016
Umbrella term formerly containing CNS neuroblastoma, ependymoblastoma, medulloepithelioma, & ETANTR
Now molecularly & genetically distinct entities considered under embryonal tumors (all WHO grade IV) include
ETMR, C19MC altered
CNS embryonal tumor, not otherwise specified (NOS)
CNS Neuroblastoma
Medulloepithelioma
Medulloblastoma
Atypical teratoid/rhabdoid tumor
IMAGING
General Features
Radiographic Findings
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
Image Interpretation Pearls
Selected References
Dangouloff-Ros V et al: CT and multimodal MR imaging features of embryonal tumors with multilayered rosettes in children. AJNR Am J Neuroradiol. 40(4):732-6, 2019
Hwang EI et al: Extensive molecular and clinical heterogeneity in patients with histologically diagnosed CNS-PNET treated as a single entity: a report from the Children's Oncology Group randomized ACNS0332 trial. J Clin Oncol. JCO2017764720, 2018
Shih RY et al: Embryonal tumors of the central nervous system: from the radiologic pathology archives. Radiographics. 38(2):525-41, 2018
Wang B et al: Embryonal tumor with multilayered rosettes, C19MC-altered: clinical, pathological, and neuroimaging findings. J Neuroimaging. 28(5):483-9, 2018
Louis DN et al: The 2016 World Health Organization classification of tumors of the central nervous system: a summary. Acta Neuropathol. 131(6):803-20, 2016
Sturm D et al: New brain tumor entities emerge from molecular classification of CNS-PNETs. Cell. 164(5):1060-72, 2016
Biswas A et al: Treatment outcome and patterns of failure in patients of non-pineal supratentorial primitive neuroectodermal tumor: review of literature and clinical experience form a regional cancer center in north India. Acta Neurochir (Wien). 157(7):1251-66, 2015
Jakacki RI et al: Outcome and prognostic factors for children with supratentorial primitive neuroectodermal tumors treated with carboplatin during radiotherapy: a report from the Children's Oncology Group. Pediatr Blood Cancer. 62(5):776-83, 2015
Nowak J et al: Systematic comparison of MRI findings in pediatric ependymoblastoma with ependymoma and CNS primitive neuroectodermal tumor not otherwise specified. Neuro Oncol. 17(8):1157-65, 2015
Phillips J et al: Practical molecular pathology and histopathology of embryonal tumors. Surg Pathol Clin. 8(1):73-88, 2015
Lester RA et al: Clinical outcomes of children and adults with central nervous system primitive neuroectodermal tumor. J Neurooncol. 120(2):371-9, 2014
De Braganca KC et al: Treatment options for medulloblastoma and CNS primitive neuroectodermal tumor (PNET). Curr Treat Options Neurol. 15(5):593-606, 2013
Perreault S et al: Relapse patterns in pediatric embryonal central nervous system tumors. J Neurooncol. 115(2):209-15, 2013
von Bueren AO: CNS PNET molecular subgroups with distinct clinical features. Lancet Oncol. 13(8):753-4, 2012
Behdad A et al: Central nervous system primitive neuroectodermal tumors: a clinicopathologic and genetic study of 33 cases. Brain Pathol. 20(2):441-50, 2010
Pfister SM et al: Molecular diagnostics of CNS embryonal tumors. Acta Neuropathol. 120(5):553-66, 2010
Johnston DL et al: Supratentorial primitive neuroectodermal tumors: a Canadian pediatric brain tumor consortium report. J Neurooncol. 86(1):101-8, 2008
Chawla A et al: Paediatric PNET: pre-surgical MRI features. Clin Radiol. 62(1):43-52, 2007
Pfister S et al: Supratentorial primitive neuroectodermal tumors of the central nervous system frequently harbor deletions of the CDKN2A locus and other genomic aberrations distinct from medulloblastomas. Genes Chromosomes Cancer. 46(9):839-51, 2007
Inda MM et al: Genetic heterogeneity in supratentorial and infratentorial primitive neuroectodermal tumours of the central nervous system. Histopathology. 47(6):631-7, 2005
Pomeroy SL et al: Prediction of central nervous system embryonal tumour outcome based on gene expression. Nature. 415(6870):436-42, 2002
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