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Pediatrics
Diagnosis
Spine
Neoplasms
Spinal Cord Ependymoma
Spinal Cord Ependymoma
Blaise V. Jones, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Definitions

              • Spinal cord neoplasm arising from ependymal cells

            IMAGING

            • General Features

              • Radiographic Findings

                • CT Findings

                  • MR Findings

                    • Nonvascular Interventions

                      • 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

                                          • Image Interpretation Pearls

                                            Selected References

                                            1. Han Z et al: Prognostic value of H3K27me3 in children with ependymoma. Pediatr Blood Cancer. 67(3):e28121, 2020
                                            2. Abdallah A et al: Long-term surgical resection outcomes of pediatric myxopapillary ependymoma: experience of two centers and brief literature review. World Neurosurg. 136:e245-61, 2019
                                            3. Swanson AA et al: Spinal cord ependymomas with MYCN amplification show aggressive clinical behavior. J Neuropathol Exp Neurol. 78(9):791-7, 2019
                                            4. Celano E et al: Spinal cord ependymoma: a review of the literature and case series of ten patients. J Neurooncol. 128(3):377-86, 2016
                                            5. Lin Y et al: Treatment of pediatric grade II spinal ependymomas: a population-based study. J Neurosurg Pediatr. 15(3):243-9, 2015
                                            6. Severino M et al: Intradural extramedullary ependymoma with leptomeningeal dissemination: the first case report in a child and literature review. World Neurosurg. 84(3):865.e13-9, 2015
                                            7. Arima H et al: Feasibility of a novel diagnostic chart of intramedullary spinal cord tumors in magnetic resonance imaging. Spinal Cord. 52(10):769-73, 2014
                                            8. Cimino PJ et al: Myxopapillary ependymoma in children: a study of 11 cases and a comparison with the adult experience. Pediatr Blood Cancer. 61(11):1969-71, 2014
                                            9. Lundar T et al: Pediatric spinal ependymomas: an unpredictable and puzzling disease. Long-term follow-up of a single consecutive institutional series of ten patients. Childs Nerv Syst. 30(12):2083-8, 2014
                                            10. Safaee M et al: Surgical outcomes in spinal cord ependymomas and the importance of extent of resection in children and young adults. J Neurosurg Pediatr. 13(4):393-9, 2014
                                            11. Shirasawa H et al: Pediatric myxopapillary ependymoma treated with subtotal resection and radiation therapy: a case report and review of the literature. Spinal Cord. 52 Suppl 2:S18-20, 2014
                                            12. Yang T et al: Clinical features and long-term outcomes of intraspinal ependymomas in pediatric patients. Childs Nerv Syst. 30(12):2073-81, 2014
                                            13. Oh MC et al: Spinal ependymomas: benefits of extent of resection for different histological grades. J Clin Neurosci. 20(10):1390-7, 2013
                                            14. Karikari IO et al: Impact of tumor histology on resectability and neurological outcome in primary intramedullary spinal cord tumors: a single-center experience with 102 patients. Neurosurgery. 68(1):188-97; discussion 197, 2011
                                            15. Plotkin SR et al: Spinal ependymomas in neurofibromatosis Type 2: a retrospective analysis of 55 patients. J Neurosurg Spine. 14(4):543-7, 2011
                                            16. Benesch M et al: Ependymoma of the spinal cord in children and adolescents: a retrospective series from the HIT database. J Neurosurg Pediatr. 6(2):137-44, 2010
                                            17. Gepp Rde A et al: Intramedullary tumors in children: analysis of 24 operated cases. Arq Neuropsiquiatr. 68(3):396-9, 2010
                                            18. Hsu W et al: Surgical considerations of spinal ependymomas in the pediatric population. Childs Nerv Syst. 25(10):1253-9, 2009
                                            19. Robles SG et al: Intradural extramedullary spinal ependymoma: a benign pathology? Spine (Phila Pa 1976). 30(9):E251-4, 2005
                                            20. Sun B et al: MRI features of intramedullary spinal cord ependymomas. J Neuroimaging. 13(4):346-51, 2003
                                            21. Carter M et al: Genetic abnormalities detected in ependymomas by comparative genomic hybridisation. Br J Cancer. 86(6):929-39, 2002
                                            22. Jeuken JW et al: Correlation between localization, age, and chromosomal imbalances in ependymal tumours as detected by CGH. J Pathol. 197(2):238-44, 2002
                                            23. Hirose Y et al: Chromosomal abnormalities subdivide ependymal tumors into clinically relevant groups. Am J Pathol. 158(3):1137-43, 2001
                                            24. Koeller KK et al: Neoplasms of the spinal cord and filum terminale: radiologic-pathologic correlation. Radiographics. 20(6):1721-49, 2000
                                            25. Miyazawa N et al: MRI at 1.5 T of intramedullary ependymoma and classification of pattern of contrast enhancement. Neuroradiology. 42(11):828-32, 2000
                                            26. Wiestler OD et al: Ependymoma. In Kleihues P et al: Pathology & Genetics of Tumors of the Central Nervous System. Lyon: IARC Press. 72-7, 2000
                                            27. Mazewski C et al: Karyotype studies in 18 ependymomas with literature review of 107 cases. Cancer Genet Cytogenet. 113(1):1-8, 1999
                                            28. Kahan H et al: MR characteristics of histopathologic subtypes of spinal ependymoma. AJNR Am J Neuroradiol. 17(1):143-50, 1996
                                            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

                                                      TERMINOLOGY

                                                      • Definitions

                                                        • Spinal cord neoplasm arising from ependymal cells

                                                      IMAGING

                                                      • General Features

                                                        • Radiographic Findings

                                                          • CT Findings

                                                            • MR Findings

                                                              • Nonvascular Interventions

                                                                • 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

                                                                                    • Image Interpretation Pearls

                                                                                      Selected References

                                                                                      1. Han Z et al: Prognostic value of H3K27me3 in children with ependymoma. Pediatr Blood Cancer. 67(3):e28121, 2020
                                                                                      2. Abdallah A et al: Long-term surgical resection outcomes of pediatric myxopapillary ependymoma: experience of two centers and brief literature review. World Neurosurg. 136:e245-61, 2019
                                                                                      3. Swanson AA et al: Spinal cord ependymomas with MYCN amplification show aggressive clinical behavior. J Neuropathol Exp Neurol. 78(9):791-7, 2019
                                                                                      4. Celano E et al: Spinal cord ependymoma: a review of the literature and case series of ten patients. J Neurooncol. 128(3):377-86, 2016
                                                                                      5. Lin Y et al: Treatment of pediatric grade II spinal ependymomas: a population-based study. J Neurosurg Pediatr. 15(3):243-9, 2015
                                                                                      6. Severino M et al: Intradural extramedullary ependymoma with leptomeningeal dissemination: the first case report in a child and literature review. World Neurosurg. 84(3):865.e13-9, 2015
                                                                                      7. Arima H et al: Feasibility of a novel diagnostic chart of intramedullary spinal cord tumors in magnetic resonance imaging. Spinal Cord. 52(10):769-73, 2014
                                                                                      8. Cimino PJ et al: Myxopapillary ependymoma in children: a study of 11 cases and a comparison with the adult experience. Pediatr Blood Cancer. 61(11):1969-71, 2014
                                                                                      9. Lundar T et al: Pediatric spinal ependymomas: an unpredictable and puzzling disease. Long-term follow-up of a single consecutive institutional series of ten patients. Childs Nerv Syst. 30(12):2083-8, 2014
                                                                                      10. Safaee M et al: Surgical outcomes in spinal cord ependymomas and the importance of extent of resection in children and young adults. J Neurosurg Pediatr. 13(4):393-9, 2014
                                                                                      11. Shirasawa H et al: Pediatric myxopapillary ependymoma treated with subtotal resection and radiation therapy: a case report and review of the literature. Spinal Cord. 52 Suppl 2:S18-20, 2014
                                                                                      12. Yang T et al: Clinical features and long-term outcomes of intraspinal ependymomas in pediatric patients. Childs Nerv Syst. 30(12):2073-81, 2014
                                                                                      13. Oh MC et al: Spinal ependymomas: benefits of extent of resection for different histological grades. J Clin Neurosci. 20(10):1390-7, 2013
                                                                                      14. Karikari IO et al: Impact of tumor histology on resectability and neurological outcome in primary intramedullary spinal cord tumors: a single-center experience with 102 patients. Neurosurgery. 68(1):188-97; discussion 197, 2011
                                                                                      15. Plotkin SR et al: Spinal ependymomas in neurofibromatosis Type 2: a retrospective analysis of 55 patients. J Neurosurg Spine. 14(4):543-7, 2011
                                                                                      16. Benesch M et al: Ependymoma of the spinal cord in children and adolescents: a retrospective series from the HIT database. J Neurosurg Pediatr. 6(2):137-44, 2010
                                                                                      17. Gepp Rde A et al: Intramedullary tumors in children: analysis of 24 operated cases. Arq Neuropsiquiatr. 68(3):396-9, 2010
                                                                                      18. Hsu W et al: Surgical considerations of spinal ependymomas in the pediatric population. Childs Nerv Syst. 25(10):1253-9, 2009
                                                                                      19. Robles SG et al: Intradural extramedullary spinal ependymoma: a benign pathology? Spine (Phila Pa 1976). 30(9):E251-4, 2005
                                                                                      20. Sun B et al: MRI features of intramedullary spinal cord ependymomas. J Neuroimaging. 13(4):346-51, 2003
                                                                                      21. Carter M et al: Genetic abnormalities detected in ependymomas by comparative genomic hybridisation. Br J Cancer. 86(6):929-39, 2002
                                                                                      22. Jeuken JW et al: Correlation between localization, age, and chromosomal imbalances in ependymal tumours as detected by CGH. J Pathol. 197(2):238-44, 2002
                                                                                      23. Hirose Y et al: Chromosomal abnormalities subdivide ependymal tumors into clinically relevant groups. Am J Pathol. 158(3):1137-43, 2001
                                                                                      24. Koeller KK et al: Neoplasms of the spinal cord and filum terminale: radiologic-pathologic correlation. Radiographics. 20(6):1721-49, 2000
                                                                                      25. Miyazawa N et al: MRI at 1.5 T of intramedullary ependymoma and classification of pattern of contrast enhancement. Neuroradiology. 42(11):828-32, 2000
                                                                                      26. Wiestler OD et al: Ependymoma. In Kleihues P et al: Pathology & Genetics of Tumors of the Central Nervous System. Lyon: IARC Press. 72-7, 2000
                                                                                      27. Mazewski C et al: Karyotype studies in 18 ependymomas with literature review of 107 cases. Cancer Genet Cytogenet. 113(1):1-8, 1999
                                                                                      28. Kahan H et al: MR characteristics of histopathologic subtypes of spinal ependymoma. AJNR Am J Neuroradiol. 17(1):143-50, 1996