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Embryonal Tumors With Multilayered Rosettes
Luke L. Linscott, MD
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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

                                            1. 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
                                            2. 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
                                            3. Shih RY et al: Embryonal tumors of the central nervous system: from the radiologic pathology archives. Radiographics. 38(2):525-41, 2018
                                            4. Wang B et al: Embryonal tumor with multilayered rosettes, C19MC-altered: clinical, pathological, and neuroimaging findings. J Neuroimaging. 28(5):483-9, 2018
                                            5. 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
                                            6. Sturm D et al: New brain tumor entities emerge from molecular classification of CNS-PNETs. Cell. 164(5):1060-72, 2016
                                            7. 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
                                            8. 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
                                            9. 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
                                            10. Phillips J et al: Practical molecular pathology and histopathology of embryonal tumors. Surg Pathol Clin. 8(1):73-88, 2015
                                            11. Lester RA et al: Clinical outcomes of children and adults with central nervous system primitive neuroectodermal tumor. J Neurooncol. 120(2):371-9, 2014
                                            12. De Braganca KC et al: Treatment options for medulloblastoma and CNS primitive neuroectodermal tumor (PNET). Curr Treat Options Neurol. 15(5):593-606, 2013
                                            13. Perreault S et al: Relapse patterns in pediatric embryonal central nervous system tumors. J Neurooncol. 115(2):209-15, 2013
                                            14. von Bueren AO: CNS PNET molecular subgroups with distinct clinical features. Lancet Oncol. 13(8):753-4, 2012
                                            15. 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
                                            16. Pfister SM et al: Molecular diagnostics of CNS embryonal tumors. Acta Neuropathol. 120(5):553-66, 2010
                                            17. Johnston DL et al: Supratentorial primitive neuroectodermal tumors: a Canadian pediatric brain tumor consortium report. J Neurooncol. 86(1):101-8, 2008
                                            18. Chawla A et al: Paediatric PNET: pre-surgical MRI features. Clin Radiol. 62(1):43-52, 2007
                                            19. 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
                                            20. Inda MM et al: Genetic heterogeneity in supratentorial and infratentorial primitive neuroectodermal tumours of the central nervous system. Histopathology. 47(6):631-7, 2005
                                            21. Pomeroy SL et al: Prediction of central nervous system embryonal tumour outcome based on gene expression. Nature. 415(6870):436-42, 2002
                                            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

                                                      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

                                                                                      1. 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
                                                                                      2. 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
                                                                                      3. Shih RY et al: Embryonal tumors of the central nervous system: from the radiologic pathology archives. Radiographics. 38(2):525-41, 2018
                                                                                      4. Wang B et al: Embryonal tumor with multilayered rosettes, C19MC-altered: clinical, pathological, and neuroimaging findings. J Neuroimaging. 28(5):483-9, 2018
                                                                                      5. 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
                                                                                      6. Sturm D et al: New brain tumor entities emerge from molecular classification of CNS-PNETs. Cell. 164(5):1060-72, 2016
                                                                                      7. 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
                                                                                      8. 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
                                                                                      9. 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
                                                                                      10. Phillips J et al: Practical molecular pathology and histopathology of embryonal tumors. Surg Pathol Clin. 8(1):73-88, 2015
                                                                                      11. Lester RA et al: Clinical outcomes of children and adults with central nervous system primitive neuroectodermal tumor. J Neurooncol. 120(2):371-9, 2014
                                                                                      12. De Braganca KC et al: Treatment options for medulloblastoma and CNS primitive neuroectodermal tumor (PNET). Curr Treat Options Neurol. 15(5):593-606, 2013
                                                                                      13. Perreault S et al: Relapse patterns in pediatric embryonal central nervous system tumors. J Neurooncol. 115(2):209-15, 2013
                                                                                      14. von Bueren AO: CNS PNET molecular subgroups with distinct clinical features. Lancet Oncol. 13(8):753-4, 2012
                                                                                      15. 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
                                                                                      16. Pfister SM et al: Molecular diagnostics of CNS embryonal tumors. Acta Neuropathol. 120(5):553-66, 2010
                                                                                      17. Johnston DL et al: Supratentorial primitive neuroectodermal tumors: a Canadian pediatric brain tumor consortium report. J Neurooncol. 86(1):101-8, 2008
                                                                                      18. Chawla A et al: Paediatric PNET: pre-surgical MRI features. Clin Radiol. 62(1):43-52, 2007
                                                                                      19. 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
                                                                                      20. Inda MM et al: Genetic heterogeneity in supratentorial and infratentorial primitive neuroectodermal tumours of the central nervous system. Histopathology. 47(6):631-7, 2005
                                                                                      21. Pomeroy SL et al: Prediction of central nervous system embryonal tumour outcome based on gene expression. Nature. 415(6870):436-42, 2002