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Posterior Fossa Ependymoma
Posterior Fossa Ependymoma
Luke L. Linscott, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Definitions

                • Posterior fossa ependymoma (PF-EPN)
                  • Slow-growing tumor of ependymal cells

              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. 28(3):318-27, 2020
                                                2. Quon JL et al: Deep learning for pediatric posterior fossa tumor detection and classification: a multi-institutional study. AJNR Am J Neuroradiol. 41(9):1718-25, 2020
                                                3. Takeishi Y et al: Advantage of high b value diffusion-weighted imaging for differentiation of common pediatric brain tumors in posterior fossa. Eur J Radiol. 128:108983, 2020
                                                4. Zhou H et al: Automatic machine learning to differentiate pediatric posterior fossa tumors on routine MR imaging. AJNR Am J Neuroradiol. 41(7):1279-85, 2020
                                                5. 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
                                                6. Ma SC et al: Clinical characteristics and prognostic factors of treatment in pediatric posterior cranial fossa ependymoma. Pediatr Neurosurg. 54(2):98-107, 2019
                                                7. Zapotocky M et al: Survival and functional outcomes of molecularly defined childhood posterior fossa ependymoma: Cure at a cost. Cancer.125(11):1867-76, 2019
                                                8. Ramaswamy V et al: Therapeutic impact of cytoreductive surgery and irradiation of posterior fossa ependymoma in the molecular era: a retrospective multicohort analysis. J Clin Oncol. 34(21):2468-77, 2016
                                                9. Sabin ND et al: Quantitative imaging analysis of posterior fossa ependymoma location in children. Childs Nerv Syst. 32(8):1441-7, 2016
                                                10. Archer TC et al: Defining the molecular landscape of ependymomas. Cancer Cell. 27(5):613-5, 2015
                                                11. Huse JT et al: The emerging molecular foundations of pediatric brain tumors. J Child Neurol. 30(13):1838-50, 2015
                                                12. Mohankumar KM et al: An in vivo screen identifies ependymoma oncogenes and tumor-suppressor genes. Nat Genet. 47(8):878-87, 2015
                                                13. 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
                                                14. Pajtler KW et al: Molecular classification of ependymal tumors across all CNS compartments, Histopathological Grades, and Age Groups. Cancer Cell. 27(5):728-43, 2015
                                                15. Thompson YY et al: Posterior fossa ependymoma: current insights. Childs Nerv Syst. 31(10):1699-706, 2015
                                                16. Mack SC et al: Epigenomic alterations define lethal CIMP-positive ependymomas of infancy. Nature. 506(7489):445-50, 2014
                                                17. Nobusawa S et al: Molecular genetics of ependymomas and pediatric diffuse gliomas: a short review. Brain Tumor Pathol. 31(4):229-33, 2014
                                                18. Merchant TE et al: Conformal radiotherapy after surgery for paediatric ependymoma: a prospective study. Lancet Oncol. 10(3):258-66, 2009
                                                19. Yuh EL et al: Imaging of ependymomas: MRI and CT. Childs Nerv Syst. 25(10):1203-13, 2009
                                                20. Schneider JF et al: Multiparametric differentiation of posterior fossa tumors in children using diffusion-weighted imaging and short echo-time 1H-MR spectroscopy. J Magn Reson Imaging. 26(6):1390-8, 2007
                                                21. Fouladi M et al: Clear cell ependymoma: a clinicopathologic and radiographic analysis of 10 patients. Cancer. 98(10):2232-44, 2003
                                                22. Good CD et al: Surveillance neuroimaging in childhood intracranial ependymoma: how effective, how often, and for how long? J Neurosurg. 94(1):27-32, 2001
                                                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

                                                            • Definitions

                                                              • Posterior fossa ependymoma (PF-EPN)
                                                                • Slow-growing tumor of ependymal cells

                                                            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. 28(3):318-27, 2020
                                                                                              2. Quon JL et al: Deep learning for pediatric posterior fossa tumor detection and classification: a multi-institutional study. AJNR Am J Neuroradiol. 41(9):1718-25, 2020
                                                                                              3. Takeishi Y et al: Advantage of high b value diffusion-weighted imaging for differentiation of common pediatric brain tumors in posterior fossa. Eur J Radiol. 128:108983, 2020
                                                                                              4. Zhou H et al: Automatic machine learning to differentiate pediatric posterior fossa tumors on routine MR imaging. AJNR Am J Neuroradiol. 41(7):1279-85, 2020
                                                                                              5. 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
                                                                                              6. Ma SC et al: Clinical characteristics and prognostic factors of treatment in pediatric posterior cranial fossa ependymoma. Pediatr Neurosurg. 54(2):98-107, 2019
                                                                                              7. Zapotocky M et al: Survival and functional outcomes of molecularly defined childhood posterior fossa ependymoma: Cure at a cost. Cancer.125(11):1867-76, 2019
                                                                                              8. Ramaswamy V et al: Therapeutic impact of cytoreductive surgery and irradiation of posterior fossa ependymoma in the molecular era: a retrospective multicohort analysis. J Clin Oncol. 34(21):2468-77, 2016
                                                                                              9. Sabin ND et al: Quantitative imaging analysis of posterior fossa ependymoma location in children. Childs Nerv Syst. 32(8):1441-7, 2016
                                                                                              10. Archer TC et al: Defining the molecular landscape of ependymomas. Cancer Cell. 27(5):613-5, 2015
                                                                                              11. Huse JT et al: The emerging molecular foundations of pediatric brain tumors. J Child Neurol. 30(13):1838-50, 2015
                                                                                              12. Mohankumar KM et al: An in vivo screen identifies ependymoma oncogenes and tumor-suppressor genes. Nat Genet. 47(8):878-87, 2015
                                                                                              13. 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
                                                                                              14. Pajtler KW et al: Molecular classification of ependymal tumors across all CNS compartments, Histopathological Grades, and Age Groups. Cancer Cell. 27(5):728-43, 2015
                                                                                              15. Thompson YY et al: Posterior fossa ependymoma: current insights. Childs Nerv Syst. 31(10):1699-706, 2015
                                                                                              16. Mack SC et al: Epigenomic alterations define lethal CIMP-positive ependymomas of infancy. Nature. 506(7489):445-50, 2014
                                                                                              17. Nobusawa S et al: Molecular genetics of ependymomas and pediatric diffuse gliomas: a short review. Brain Tumor Pathol. 31(4):229-33, 2014
                                                                                              18. Merchant TE et al: Conformal radiotherapy after surgery for paediatric ependymoma: a prospective study. Lancet Oncol. 10(3):258-66, 2009
                                                                                              19. Yuh EL et al: Imaging of ependymomas: MRI and CT. Childs Nerv Syst. 25(10):1203-13, 2009
                                                                                              20. Schneider JF et al: Multiparametric differentiation of posterior fossa tumors in children using diffusion-weighted imaging and short echo-time 1H-MR spectroscopy. J Magn Reson Imaging. 26(6):1390-8, 2007
                                                                                              21. Fouladi M et al: Clear cell ependymoma: a clinicopathologic and radiographic analysis of 10 patients. Cancer. 98(10):2232-44, 2003
                                                                                              22. Good CD et al: Surveillance neuroimaging in childhood intracranial ependymoma: how effective, how often, and for how long? J Neurosurg. 94(1):27-32, 2001