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Supratentorial Ependymoma
Luke L. Linscott, MD
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KEY FACTS

  • Imaging

    • Top Differential Diagnoses

      • Pathology

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Abbreviations

              • Supratentorial ependymoma (STE)
            • Synonyms

              • Hemispheric STE (a.k.a. brain surface ependymoma)
              • Supratentorial ectopic cortical ependymoma
              • Cortical ependymoma
            • Definitions

              • Ependymoma in supratentorial location

            IMAGING

            • General Features

              • CT Findings

                • MR 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

                                        Selected References

                                        1. Lillard JC et al: Pediatric supratentorial ependymoma: surgical, clinical, and molecular analysis. Neurosurgery. 85(1):41-9. 2018
                                        2. Nowak J et al: MRI phenotype of RELA-fused pediatric supratentorial ependymoma. Clin Neuroradiol. ePub, 2018
                                        3. Onishi S et al: RELA fusion-positive anaplastic ependymoma: molecular characterization and advanced MR imaging. Brain Tumor Pathol. 35(1):41-5, 2018
                                        4. Sun S et al: Clinical, radiological, and histological features and treatment outcomes of supratentorial extraventricular ependymoma: 14 cases from a single center. J Neurosurg. 128(5):1396-1402, 2018
                                        5. Witt DA et al: Specific expression of PD-L1 in RELA-fusion supratentorial ependymoma: implications for PD-1-targeted therapy. Pediatr Blood Cancer. 65(5):e26960, 2018
                                        6. Gessi M et al: Role of immunohistochemistry in the Identification of supratentorial C11ORF95-RELA fused ependymoma in routine neuropathology. Am J Surg Pathol. 43(1):56-63, 2017
                                        7. de Andrade FG et al: Immunohistochemical expression of cyclin D1 is higher in supratentorial ependymomas and predicts relapses in gross total resection cases. Neuropathology. 35(4):312-3, 2015
                                        8. Mohaghegh MR et al: Supratentorial cortical ependymoma: an unusual presentation of a rare tumor. Adv Biomed Res. 4:72, 2015
                                        9. 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
                                        10. Khilji MF et al: Supratentorial extraventricular anaplastic ependymoma in a child. BMJ Case Rep. 2014, 2014
                                        11. Purdy E et al: Ependymoma in children under the age of 3 years: a report from the Canadian Pediatric Brain Tumour Consortium. J Neurooncol. 117(2):359-64, 2014
                                        12. Alexiou GA et al: Supratentorial ependymomas in children: analysis of nine cases. J Pediatr Neurosci. 8(1):15-8, 2013
                                        13. Cage TA et al: A systematic review of treatment outcomes in pediatric patients with intracranial ependymomas. J Neurosurg Pediatr. 11(6):673-81, 2013
                                        14. Li JY et al: Giant cell ependymoma-report of three cases and review of the literature. Int J Clin Exp Pathol. 5(5):458-62, 2012
                                        15. Reis F et al: Supratentorial tanycytic ependymoma: an uncommon fibrillary ependymoma variant. Arq Neuropsiquiatr. 69(4):723, 2011
                                        16. Niazi TN et al: WHO Grade II and III supratentorial hemispheric ependymomas in adults: case series and review of treatment options. J Neurooncol. 91(3):323-8, 2009
                                        17. Yi W et al: Expression of leucine-rich repeats and immunoglobulin-like domains (LRIG) proteins in human ependymoma relates to tumor location, WHO grade, and patient age. Clin Neuropathol. 28(1):21-7, 2009
                                        18. Lehman NL: Central nervous system tumors with ependymal features: a broadened spectrum of primarily ependymal differentiation? J Neuropathol Exp Neurol. 67(3):177-88, 2008
                                        19. Lehman NL: Patterns of brain infiltration and secondary structure formation in supratentorial ependymal tumors. J Neuropathol Exp Neurol. 67(9):900-10, 2008
                                        20. Miyazawa T et al: Supratentorial ectopic cortical ependymoma occurring with intratumoral hemorrhage. Brain Tumor Pathol. 24(1):35-40, 2007
                                        21. Rousseau E et al: Trisomy 19 ependymoma, a newly recognized genetico-histological association, including clear cell ependymoma. Mol Cancer. 6:47, 2007
                                        22. Vinchon M et al: Supratentorial ependymoma in children. Pediatr Neurosurg. 34(2):77-87, 2001
                                        Related Anatomy
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                                        Related Differential Diagnoses
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                                        References
                                        Tables

                                        Tables

                                        KEY FACTS

                                        • Imaging

                                          • Top Differential Diagnoses

                                            • Pathology

                                              • Clinical Issues

                                                • Diagnostic Checklist

                                                  TERMINOLOGY

                                                  • Abbreviations

                                                    • Supratentorial ependymoma (STE)
                                                  • Synonyms

                                                    • Hemispheric STE (a.k.a. brain surface ependymoma)
                                                    • Supratentorial ectopic cortical ependymoma
                                                    • Cortical ependymoma
                                                  • Definitions

                                                    • Ependymoma in supratentorial location

                                                  IMAGING

                                                  • General Features

                                                    • CT Findings

                                                      • MR 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

                                                                              Selected References

                                                                              1. Lillard JC et al: Pediatric supratentorial ependymoma: surgical, clinical, and molecular analysis. Neurosurgery. 85(1):41-9. 2018
                                                                              2. Nowak J et al: MRI phenotype of RELA-fused pediatric supratentorial ependymoma. Clin Neuroradiol. ePub, 2018
                                                                              3. Onishi S et al: RELA fusion-positive anaplastic ependymoma: molecular characterization and advanced MR imaging. Brain Tumor Pathol. 35(1):41-5, 2018
                                                                              4. Sun S et al: Clinical, radiological, and histological features and treatment outcomes of supratentorial extraventricular ependymoma: 14 cases from a single center. J Neurosurg. 128(5):1396-1402, 2018
                                                                              5. Witt DA et al: Specific expression of PD-L1 in RELA-fusion supratentorial ependymoma: implications for PD-1-targeted therapy. Pediatr Blood Cancer. 65(5):e26960, 2018
                                                                              6. Gessi M et al: Role of immunohistochemistry in the Identification of supratentorial C11ORF95-RELA fused ependymoma in routine neuropathology. Am J Surg Pathol. 43(1):56-63, 2017
                                                                              7. de Andrade FG et al: Immunohistochemical expression of cyclin D1 is higher in supratentorial ependymomas and predicts relapses in gross total resection cases. Neuropathology. 35(4):312-3, 2015
                                                                              8. Mohaghegh MR et al: Supratentorial cortical ependymoma: an unusual presentation of a rare tumor. Adv Biomed Res. 4:72, 2015
                                                                              9. 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
                                                                              10. Khilji MF et al: Supratentorial extraventricular anaplastic ependymoma in a child. BMJ Case Rep. 2014, 2014
                                                                              11. Purdy E et al: Ependymoma in children under the age of 3 years: a report from the Canadian Pediatric Brain Tumour Consortium. J Neurooncol. 117(2):359-64, 2014
                                                                              12. Alexiou GA et al: Supratentorial ependymomas in children: analysis of nine cases. J Pediatr Neurosci. 8(1):15-8, 2013
                                                                              13. Cage TA et al: A systematic review of treatment outcomes in pediatric patients with intracranial ependymomas. J Neurosurg Pediatr. 11(6):673-81, 2013
                                                                              14. Li JY et al: Giant cell ependymoma-report of three cases and review of the literature. Int J Clin Exp Pathol. 5(5):458-62, 2012
                                                                              15. Reis F et al: Supratentorial tanycytic ependymoma: an uncommon fibrillary ependymoma variant. Arq Neuropsiquiatr. 69(4):723, 2011
                                                                              16. Niazi TN et al: WHO Grade II and III supratentorial hemispheric ependymomas in adults: case series and review of treatment options. J Neurooncol. 91(3):323-8, 2009
                                                                              17. Yi W et al: Expression of leucine-rich repeats and immunoglobulin-like domains (LRIG) proteins in human ependymoma relates to tumor location, WHO grade, and patient age. Clin Neuropathol. 28(1):21-7, 2009
                                                                              18. Lehman NL: Central nervous system tumors with ependymal features: a broadened spectrum of primarily ependymal differentiation? J Neuropathol Exp Neurol. 67(3):177-88, 2008
                                                                              19. Lehman NL: Patterns of brain infiltration and secondary structure formation in supratentorial ependymal tumors. J Neuropathol Exp Neurol. 67(9):900-10, 2008
                                                                              20. Miyazawa T et al: Supratentorial ectopic cortical ependymoma occurring with intratumoral hemorrhage. Brain Tumor Pathol. 24(1):35-40, 2007
                                                                              21. Rousseau E et al: Trisomy 19 ependymoma, a newly recognized genetico-histological association, including clear cell ependymoma. Mol Cancer. 6:47, 2007
                                                                              22. Vinchon M et al: Supratentorial ependymoma in children. Pediatr Neurosurg. 34(2):77-87, 2001