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Pediatrics
Diagnosis
Brain
Pathology-Based Diagnoses
Nonneoplastic Cysts and Tumor Mimics
Ependymoma
Ependymoma
Luke L. Linscott, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Definitions

                • Slow-growing tumor resembling ependymal cells
                  • Likely progenitor cell: Radial glial cell in subventricular zone
                • Other ependymal tumors genetically distinct; share some histologic features
                  • Subependymoma, myxopapillary ependymoma, anaplastic ependymoma

              IMAGING

              • General Features

                • CT Findings

                  • MR 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. Domínguez-Pinilla N et al: Evaluating the apparent diffusion coefficient in MRI studies as a means of determining paediatric brain tumour stages. Neurologia. ePub, 2015
                                              2. 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
                                              3. Thompson YY et al: Posterior fossa ependymoma: current insights. Childs Nerv Syst. 31(10):1699-706, 2015
                                              4. Liu Z et al: Supratentorial cortical ependymoma: case series and review of the literature. Neuropathology. 34(3):243-52, 2014
                                              5. Nowak J et al: MRI characteristics of ependymoblastoma: results from 22 centrally reviewed cases. AJNR Am J Neuroradiol. 35(10):1996-2001, 2014
                                              6. Ostrom QT et al: CBTRUS statistical report: primary brain and central nervous system tumors diagnosed in the United States in 2007-2011. Neuro Oncol. 16 Suppl 4:iv1-63, 2014
                                              7. Rodriguez Gutierrez D et al: Metrics and textural features of MRI diffusion to improve classification of pediatric posterior fossa tumors. AJNR Am J Neuroradiol. 35(5):1009-15, 2014
                                              8. Smith AB et al: From the radiologic pathology archives: intraventricular neoplasms: radiologic-pathologic correlation. Radiographics. 33(1):21-43, 2013
                                              9. Witt H et al: Delineation of two clinically and molecularly distinct subgroups of posterior fossa ependymoma. Cancer Cell. 20(2):143-57, 2011
                                              10. Andreiuolo F et al: Neuronal differentiation distinguishes supratentorial and infratentorial childhood ependymomas. Neuro Oncol. 12(11):1126-34, 2010
                                              11. Cuellar-Baena S et al: Comparative metabolic profiling of paediatric ependymoma, medulloblastoma and pilocytic astrocytoma. Int J Mol Med. 26(6):941-8, 2010
                                              12. U-King-Im JM et al: Posterior fossa ependymomas: new radiological classification with surgical correlation. Childs Nerv Syst. 26(12):1765-72, 2010
                                              13. Zacharoulis S et al: Treatment and outcome of children with relapsed ependymoma: a multi-institutional retrospective analysis. Childs Nerv Syst. 26(7):905-11, 2010
                                              14. Jaing TH et al: Multivariate analysis of clinical prognostic factors in children with intracranial ependymomas. J Neurooncol. 68(3):255-61, 2004
                                              15. 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
                                              16. Akyuz C et al: Intracranial ependymomas in childhood--a retrospective review of sixty-two children. Acta Oncol. 39(1):97-100, 2000
                                              17. Palma L et al: The importance of surgery in supratentorial ependymomas. Long-term survival in a series of 23 cases. Childs Nerv Syst. 16(3):170-5, 2000
                                              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

                                                            • Slow-growing tumor resembling ependymal cells
                                                              • Likely progenitor cell: Radial glial cell in subventricular zone
                                                            • Other ependymal tumors genetically distinct; share some histologic features
                                                              • Subependymoma, myxopapillary ependymoma, anaplastic ependymoma

                                                          IMAGING

                                                          • General Features

                                                            • CT Findings

                                                              • MR 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. Domínguez-Pinilla N et al: Evaluating the apparent diffusion coefficient in MRI studies as a means of determining paediatric brain tumour stages. Neurologia. ePub, 2015
                                                                                          2. 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
                                                                                          3. Thompson YY et al: Posterior fossa ependymoma: current insights. Childs Nerv Syst. 31(10):1699-706, 2015
                                                                                          4. Liu Z et al: Supratentorial cortical ependymoma: case series and review of the literature. Neuropathology. 34(3):243-52, 2014
                                                                                          5. Nowak J et al: MRI characteristics of ependymoblastoma: results from 22 centrally reviewed cases. AJNR Am J Neuroradiol. 35(10):1996-2001, 2014
                                                                                          6. Ostrom QT et al: CBTRUS statistical report: primary brain and central nervous system tumors diagnosed in the United States in 2007-2011. Neuro Oncol. 16 Suppl 4:iv1-63, 2014
                                                                                          7. Rodriguez Gutierrez D et al: Metrics and textural features of MRI diffusion to improve classification of pediatric posterior fossa tumors. AJNR Am J Neuroradiol. 35(5):1009-15, 2014
                                                                                          8. Smith AB et al: From the radiologic pathology archives: intraventricular neoplasms: radiologic-pathologic correlation. Radiographics. 33(1):21-43, 2013
                                                                                          9. Witt H et al: Delineation of two clinically and molecularly distinct subgroups of posterior fossa ependymoma. Cancer Cell. 20(2):143-57, 2011
                                                                                          10. Andreiuolo F et al: Neuronal differentiation distinguishes supratentorial and infratentorial childhood ependymomas. Neuro Oncol. 12(11):1126-34, 2010
                                                                                          11. Cuellar-Baena S et al: Comparative metabolic profiling of paediatric ependymoma, medulloblastoma and pilocytic astrocytoma. Int J Mol Med. 26(6):941-8, 2010
                                                                                          12. U-King-Im JM et al: Posterior fossa ependymomas: new radiological classification with surgical correlation. Childs Nerv Syst. 26(12):1765-72, 2010
                                                                                          13. Zacharoulis S et al: Treatment and outcome of children with relapsed ependymoma: a multi-institutional retrospective analysis. Childs Nerv Syst. 26(7):905-11, 2010
                                                                                          14. Jaing TH et al: Multivariate analysis of clinical prognostic factors in children with intracranial ependymomas. J Neurooncol. 68(3):255-61, 2004
                                                                                          15. 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
                                                                                          16. Akyuz C et al: Intracranial ependymomas in childhood--a retrospective review of sixty-two children. Acta Oncol. 39(1):97-100, 2000
                                                                                          17. Palma L et al: The importance of surgery in supratentorial ependymomas. Long-term survival in a series of 23 cases. Childs Nerv Syst. 16(3):170-5, 2000