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Other Embryonal Tumors
Anne G. Osborn, MD, FACR
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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

                                              1. Li BK et al: Pediatric embryonal brain tumors in the molecular era. Expert Rev Mol Diagn. 20(3):293-303, 2020
                                              2. de Kock L et al: An update on the central nervous system manifestations of DICER1 syndrome. Acta Neuropathol. 139(4):689-701, 2019
                                              3. Lambo S et al: The molecular landscape of ETMR at diagnosis and relapse. Nature. 576(7786):274-80, 2019
                                              4. 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
                                              5. 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
                                              6. Bouali S et al: Embryonal tumor with multilayered rosettes: illustrative case and review of the literature. Childs Nerv Syst. 34(12):2361-9, 2018
                                              7. 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
                                              8. Sin-Chan P et al: Molecular classification and management of rare pediatric embryonal brain tumors. Curr Oncol Rep. 20(9):69, 2018
                                              9. Wang B et al: Embryonal tumor with multilayered rosettes, C19MC-altered: clinical, pathological, and neuroimaging findings. J Neuroimaging. 28(5):483-9, 2018
                                              10. 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
                                              11. 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

                                                                                          1. Li BK et al: Pediatric embryonal brain tumors in the molecular era. Expert Rev Mol Diagn. 20(3):293-303, 2020
                                                                                          2. de Kock L et al: An update on the central nervous system manifestations of DICER1 syndrome. Acta Neuropathol. 139(4):689-701, 2019
                                                                                          3. Lambo S et al: The molecular landscape of ETMR at diagnosis and relapse. Nature. 576(7786):274-80, 2019
                                                                                          4. 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
                                                                                          5. 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
                                                                                          6. Bouali S et al: Embryonal tumor with multilayered rosettes: illustrative case and review of the literature. Childs Nerv Syst. 34(12):2361-9, 2018
                                                                                          7. 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
                                                                                          8. Sin-Chan P et al: Molecular classification and management of rare pediatric embryonal brain tumors. Curr Oncol Rep. 20(9):69, 2018
                                                                                          9. Wang B et al: Embryonal tumor with multilayered rosettes, C19MC-altered: clinical, pathological, and neuroimaging findings. J Neuroimaging. 28(5):483-9, 2018
                                                                                          10. 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
                                                                                          11. 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