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Ependymoma
Anne G. Osborn, MD, FACR
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Ependymoma (EPN)
                • Posterior fossa ependymoma (PF-EPN)
              • Definitions

                • Ependymomas have 9 clinically, demographically, molecularly distinct entities
                  • Divided into 3 anatomic compartments
                    • Supratentorial (ST), posterior fossa (PF), spinal (SPN)
                  • 3 subgroups occur in each compartment
                  • 2/3 of intracranial EPNs infratentorial (PF-EPN)
                    • Divided by methylation profiling into 2 main groups
                      • PF-EPN subtype A (PF-EPN-A): Sometimes called "pediatric-type"
                      • PF-EPN subtype B (PF-EPN-B): Sometimes called "adult-type"

              IMAGING

              • General Features

                • CT Findings

                  • MR Findings

                    • Angiographic Findings

                      • Nuclear Medicine 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. Dong J et al: Differentiation between ependymoma and medulloblastoma in children with radiomics approach. Acad Radiol. ePub, 2020
                                                2. Evenson M et al: Utility of copy number variants in the classification of intracranial ependymoma. Cancer Genet. 240:66-72, 2020
                                                3. Lester A et al: Intracranial ependymomas: molecular insights and translation to treatment. Brain Pathol. 30(1):3-12, 2020
                                                4. Li M et al: Ependymoma and pilocytic astrocytoma: Differentiation using radiomics approach based on machine learning. J Clin Neurosci. ePub, 2020
                                                5. Neumann JE et al: Molecular characterization of histopathological ependymoma variants. Acta Neuropathol. 139(2):305-18, 2020
                                                6. Payabvash S et al: Machine learning decision tree models for differentiation of posterior fossa tumors using diffusion histogram analysis and structural MRI findings. Front Oncol. 10:71, 2020
                                                7. Tsangaris GT et al: Proteomics of pediatric ependymomas: a review. Childs Nerv Syst. ePub, 2020
                                                8. Delgado-López PD et al: Central nervous system ependymoma: clinical implications of the new molecular classification, treatment guidelines and controversial issues. Clin Transl Oncol. 21(11):1450-63, 2019
                                                9. Jünger ST et al: Improved risk-stratification for posterior fossa ependymoma of childhood considering clinical, histological and genetic features - a retrospective analysis of the HIT ependymoma trial cohort. Acta Neuropathol Commun. 7(1):181, 2019
                                                10. Ma SC et al: Clinical characteristics and prognostic factors of treatment in pediatric posterior cranial fossa ependymoma. Pediatr Neurosurg. 54(2):98-107, 2019
                                                11. Sasaki A et al: Review of ependymomas: assessment of consensus in pathological diagnosis and correlations with genetic profiles and outcome. Brain Tumor Pathol. 36(2):92-101, 2019
                                                12. Cavalli FMG et al: Heterogeneity within the PF-EPN-B ependymoma subgroup. Acta Neuropathol. 136(2):227-37, 2018
                                                13. D'Arco F et al: Differential diagnosis of posterior fossa tumours in children: new insights. Pediatr Radiol. 48(13):1955-63, 2018
                                                14. Pajtler KW et al: Molecular heterogeneity and CXorf67 alterations in posterior fossa group A (PFA) ependymomas. Acta Neuropathol. 136(2):211-26, 2018
                                                15. Brandão LA et al: Posterior fossa tumors. Neuroimaging Clin N Am. 27(1):1-37, 2017
                                                16. Pajtler KW et al: The current consensus on the clinical management of intracranial ependymoma and its distinct molecular variants. Acta Neuropathol. 133(1):5-12, 2017
                                                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

                                                              • Ependymoma (EPN)
                                                              • Posterior fossa ependymoma (PF-EPN)
                                                            • Definitions

                                                              • Ependymomas have 9 clinically, demographically, molecularly distinct entities
                                                                • Divided into 3 anatomic compartments
                                                                  • Supratentorial (ST), posterior fossa (PF), spinal (SPN)
                                                                • 3 subgroups occur in each compartment
                                                                • 2/3 of intracranial EPNs infratentorial (PF-EPN)
                                                                  • Divided by methylation profiling into 2 main groups
                                                                    • PF-EPN subtype A (PF-EPN-A): Sometimes called "pediatric-type"
                                                                    • PF-EPN subtype B (PF-EPN-B): Sometimes called "adult-type"

                                                            IMAGING

                                                            • General Features

                                                              • CT Findings

                                                                • MR Findings

                                                                  • Angiographic Findings

                                                                    • Nuclear Medicine 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. Dong J et al: Differentiation between ependymoma and medulloblastoma in children with radiomics approach. Acad Radiol. ePub, 2020
                                                                                              2. Evenson M et al: Utility of copy number variants in the classification of intracranial ependymoma. Cancer Genet. 240:66-72, 2020
                                                                                              3. Lester A et al: Intracranial ependymomas: molecular insights and translation to treatment. Brain Pathol. 30(1):3-12, 2020
                                                                                              4. Li M et al: Ependymoma and pilocytic astrocytoma: Differentiation using radiomics approach based on machine learning. J Clin Neurosci. ePub, 2020
                                                                                              5. Neumann JE et al: Molecular characterization of histopathological ependymoma variants. Acta Neuropathol. 139(2):305-18, 2020
                                                                                              6. Payabvash S et al: Machine learning decision tree models for differentiation of posterior fossa tumors using diffusion histogram analysis and structural MRI findings. Front Oncol. 10:71, 2020
                                                                                              7. Tsangaris GT et al: Proteomics of pediatric ependymomas: a review. Childs Nerv Syst. ePub, 2020
                                                                                              8. Delgado-López PD et al: Central nervous system ependymoma: clinical implications of the new molecular classification, treatment guidelines and controversial issues. Clin Transl Oncol. 21(11):1450-63, 2019
                                                                                              9. Jünger ST et al: Improved risk-stratification for posterior fossa ependymoma of childhood considering clinical, histological and genetic features - a retrospective analysis of the HIT ependymoma trial cohort. Acta Neuropathol Commun. 7(1):181, 2019
                                                                                              10. Ma SC et al: Clinical characteristics and prognostic factors of treatment in pediatric posterior cranial fossa ependymoma. Pediatr Neurosurg. 54(2):98-107, 2019
                                                                                              11. Sasaki A et al: Review of ependymomas: assessment of consensus in pathological diagnosis and correlations with genetic profiles and outcome. Brain Tumor Pathol. 36(2):92-101, 2019
                                                                                              12. Cavalli FMG et al: Heterogeneity within the PF-EPN-B ependymoma subgroup. Acta Neuropathol. 136(2):227-37, 2018
                                                                                              13. D'Arco F et al: Differential diagnosis of posterior fossa tumours in children: new insights. Pediatr Radiol. 48(13):1955-63, 2018
                                                                                              14. Pajtler KW et al: Molecular heterogeneity and CXorf67 alterations in posterior fossa group A (PFA) ependymomas. Acta Neuropathol. 136(2):211-26, 2018
                                                                                              15. Brandão LA et al: Posterior fossa tumors. Neuroimaging Clin N Am. 27(1):1-37, 2017
                                                                                              16. Pajtler KW et al: The current consensus on the clinical management of intracranial ependymoma and its distinct molecular variants. Acta Neuropathol. 133(1):5-12, 2017