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Subependymoma
Karen L. Salzman, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Synonyms

                • Older literature: Subependymal glomerulate astrocytoma, subependymal astrocytoma, subependymal mixed glioma
              • Definitions

                • Rare, benign, well-differentiated, intraventricular ependymal tumor, often attached to ventricular wall

              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. Pontillo G et al: Is this truly a "leave-me-alone" lesion? An unusual case of multiple ring-shaped lateral ventricular nodules. World Neurosurg. 136:32-6, 2020
                                              2. Wu L et al: Subependymoma of the conus medullaris with cystic formation: case report and a literature review. World Neurosurg. 137:235-8, 2020
                                              3. Klotz E et al: Minimizing cortical disturbance to access ventricular subependymoma - a novel approach utilizing spinal minimally invasive tubular retractor system. Surg Neurol Int. 10:95, 2019
                                              4. Mikula AL et al: Subependymoma involving multiple spinal cord levels: A clinicopathological case series with chromosomal microarray analysis. Neuropathology. 39(2):97-105, 2019
                                              5. Yao K et al: Detection of H3K27M mutation in cases of brain stem subependymoma. Hum Pathol. 84:262-9, 2019
                                              6. Moinuddin FM et al: Bilateral lateral ventricular subependymoma with extensive multiplicity presenting with hemorrhage. Neuroradiol J. 31(1):27-31, 2018
                                              7. Muly S et al: MRI of intracranial intraventricular lesions. Clin Imaging. 52:226-39, 2018
                                              8. Varma A et al: Surgical management and long-term outcome of intracranial subependymoma. Acta Neurochir (Wien). 160(9):1793-9, 2018
                                              9. Leeper H et al: Recent advances in the classification and treatment of ependymomas. Curr Treat Options Oncol. 18(9):55, 2017
                                              10. Nguyen HS et al: Intracranial subependymoma: a SEER analysis 2004-2013. World Neurosurg. 101:599-605, 2017
                                              11. Ueda F et al: MR spectroscopy to distinguish between supratentorial intraventricular subependymoma and central neurocytoma. Magn Reson Med Sci. 16(3):223-30 2016
                                              12. Louis DN et al: WHO Classification of Tumours of the Central Nervous System: Subependymoma. Lyon: IARC Press. 102-3, 2016
                                              13. Zhou S et al: Neuroradiological features of cervical and cervicothoracic intraspinal subependymomas: a study of five cases. Clin Radiol. 71(5):499.e9-15, 2016
                                              14. Bi Z et al: Clinical, radiological, and pathological features in 43 cases of intracranial subependymoma. J Neurosurg. 122(1):49-60, 2015
                                              15. Arvanitis LD et al: A 40-year-old male with an intraventricular tumor. Combined tanycytic ependymoma and subependymoma. Brain Pathol. 23(3):359-60, 2013
                                              16. Hou Z et al: Clinical features and management of intracranial subependymomas in children. J Clin Neurosci. 20(1):84-8, 2013
                                              17. Iwasaki M et al: Thoracolumbar intramedullary subependymoma with multiple cystic formation: a case report and review. Eur Spine J. 22 Suppl 3:S317-20, 2013
                                              18. Smith AB et al: From the radiologic pathology archives: intraventricular neoplasms: radiologic-pathologic correlation. Radiographics. 33(1):21-43, 2013
                                              19. Cunha AM et al: Cerebellopontine angle subependymoma without fourth ventricle extension: an uncommon tumor in a rare location. Neuropathology. 32(2):164-70, 2012
                                              20. Jain A et al: Subependymoma: clinical features and surgical outcomes. Neurol Res. 34(7):677-84, 2012
                                              21. Koral K et al: Subependymoma of the cerebellopontine angle and prepontine cistern in a 15-year-old adolescent boy. AJNR Am J Neuroradiol. 29(1):190-1, 2008
                                              22. Romoli S et al: Unusual exophytic subependymoma in the bulbo-cerebellar angle. Case report. J Neurosurg Sci. 51(2):81-4, 2007
                                              23. Rushing EJ et al: Subependymoma revisited: clinicopathological evaluation of 83 cases. J Neurooncol. 85(3):297-305, 2007
                                              24. Ragel BT et al: Subependymomas: an analysis of clinical and imaging features. Neurosurgery. 58(5):881-90; discussion 881-90, 2006
                                              25. Rath TJ et al: Massive symptomatic subependymoma of the lateral ventricles: case report and review of the literature. Neuroradiology. 47(3):183-8, 2005
                                              26. Shuangshoti S et al: Supratentorial extraventricular ependymal neoplasms: a clinicopathologic study of 32 patients. Cancer. 103(12):2598-605, 2005
                                              27. Kim HC et al: Subependymoma in the third ventricle in a child. Clin Imaging. 28(5):381-4, 2004
                                              28. Im SH et al: Clinicopathological study of seven cases of symptomatic supratentorial subependymoma. J Neurooncol. 61(1):57-67, 2003
                                              29. Seol HJ et al: A case of recurrent subependymoma with subependymal seeding: case report. J Neurooncol. 62(3):315-20, 2003
                                              30. Burger PC et al: Surgical pathology of the nervous system and its coverings: The Brain: Tumors. 4th ed. Philadelphia: Churchill Livingstone. 250-4, 2002
                                              31. Ironside JW et al: Diagnostic pathology of nervous system tumours: Ependymal and choroid plexus tumors. 1st ed. Edinburgh: Churchill Livingstone. 145-83, 2002
                                              32. Nishio S et al: Tumours around the foramen of Monro: clinical and neuroimaging features and their differential diagnosis. J Clin Neurosci. 9(2):137-41, 2002
                                              33. Fontenele GI et al: Symptomatic child case of subependymoma in the fourth ventricle without hydrocephalus. Radiat Med. 19(1):37-42, 2001
                                              34. Wiestler OD et al: Pathology and genetics of tumours of the nervous system: Subependymoma. Lyon: IARC Press. 80-1, 2000
                                              35. Maiuri F et al: Symptomatic subependymomas of the lateral ventricles. Report of eight cases. Clin Neurol Neurosurg. 99(1):17-22, 1997
                                              36. Mineura K et al: Subependymoma of the septum pellucidum: characterization by PET. J Neurooncol. 32(2):143-7, 1997
                                              37. Jallo GI et al: Intramedullary subependymoma of the spinal cord. Neurosurgery. 38(2):251-7, 1996
                                              38. Chiechi MV et al: Intracranial subependymomas: CT and MR imaging features in 24 cases. AJR Am J Roentgenol. 165(5):1245-50, 1995
                                              39. Furie DM et al: Supratentorial ependymomas and subependymomas: CT and MR appearance. J Comput Assist Tomogr. 19(4):518-26, 1995
                                              40. Hoeffel C et al: MR manifestations of subependymomas. AJNR Am J Neuroradiol. 16(10):2121-9, 1995
                                              41. Silverstein JE et al: MRI of intracranial subependymomas. J Comput Assist Tomogr. 19(2):264-7, 1995
                                              42. Iqbal Z et al: Subependymoma of the lateral ventricle: case report and literature review. Br J Neurosurg. 8(1):83-5, 1994
                                              43. Ryken TC et al: Familial occurrence of subependymoma. Report of two cases. J Neurosurg. 80(6):1108-11, 1994
                                              44. Lindboe CF et al: Hemorrhage in a highly vascularized subependymoma of the septum pellucidum: case report. Neurosurgery. 31(4):741-5, 1992
                                              45. Spoto GP et al: Intracranial ependymoma and subependymoma: MR manifestations. AJNR Am J Neuroradiol. 11(1):83-91, 1990
                                              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

                                                          • Synonyms

                                                            • Older literature: Subependymal glomerulate astrocytoma, subependymal astrocytoma, subependymal mixed glioma
                                                          • Definitions

                                                            • Rare, benign, well-differentiated, intraventricular ependymal tumor, often attached to ventricular wall

                                                          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. Pontillo G et al: Is this truly a "leave-me-alone" lesion? An unusual case of multiple ring-shaped lateral ventricular nodules. World Neurosurg. 136:32-6, 2020
                                                                                          2. Wu L et al: Subependymoma of the conus medullaris with cystic formation: case report and a literature review. World Neurosurg. 137:235-8, 2020
                                                                                          3. Klotz E et al: Minimizing cortical disturbance to access ventricular subependymoma - a novel approach utilizing spinal minimally invasive tubular retractor system. Surg Neurol Int. 10:95, 2019
                                                                                          4. Mikula AL et al: Subependymoma involving multiple spinal cord levels: A clinicopathological case series with chromosomal microarray analysis. Neuropathology. 39(2):97-105, 2019
                                                                                          5. Yao K et al: Detection of H3K27M mutation in cases of brain stem subependymoma. Hum Pathol. 84:262-9, 2019
                                                                                          6. Moinuddin FM et al: Bilateral lateral ventricular subependymoma with extensive multiplicity presenting with hemorrhage. Neuroradiol J. 31(1):27-31, 2018
                                                                                          7. Muly S et al: MRI of intracranial intraventricular lesions. Clin Imaging. 52:226-39, 2018
                                                                                          8. Varma A et al: Surgical management and long-term outcome of intracranial subependymoma. Acta Neurochir (Wien). 160(9):1793-9, 2018
                                                                                          9. Leeper H et al: Recent advances in the classification and treatment of ependymomas. Curr Treat Options Oncol. 18(9):55, 2017
                                                                                          10. Nguyen HS et al: Intracranial subependymoma: a SEER analysis 2004-2013. World Neurosurg. 101:599-605, 2017
                                                                                          11. Ueda F et al: MR spectroscopy to distinguish between supratentorial intraventricular subependymoma and central neurocytoma. Magn Reson Med Sci. 16(3):223-30 2016
                                                                                          12. Louis DN et al: WHO Classification of Tumours of the Central Nervous System: Subependymoma. Lyon: IARC Press. 102-3, 2016
                                                                                          13. Zhou S et al: Neuroradiological features of cervical and cervicothoracic intraspinal subependymomas: a study of five cases. Clin Radiol. 71(5):499.e9-15, 2016
                                                                                          14. Bi Z et al: Clinical, radiological, and pathological features in 43 cases of intracranial subependymoma. J Neurosurg. 122(1):49-60, 2015
                                                                                          15. Arvanitis LD et al: A 40-year-old male with an intraventricular tumor. Combined tanycytic ependymoma and subependymoma. Brain Pathol. 23(3):359-60, 2013
                                                                                          16. Hou Z et al: Clinical features and management of intracranial subependymomas in children. J Clin Neurosci. 20(1):84-8, 2013
                                                                                          17. Iwasaki M et al: Thoracolumbar intramedullary subependymoma with multiple cystic formation: a case report and review. Eur Spine J. 22 Suppl 3:S317-20, 2013
                                                                                          18. Smith AB et al: From the radiologic pathology archives: intraventricular neoplasms: radiologic-pathologic correlation. Radiographics. 33(1):21-43, 2013
                                                                                          19. Cunha AM et al: Cerebellopontine angle subependymoma without fourth ventricle extension: an uncommon tumor in a rare location. Neuropathology. 32(2):164-70, 2012
                                                                                          20. Jain A et al: Subependymoma: clinical features and surgical outcomes. Neurol Res. 34(7):677-84, 2012
                                                                                          21. Koral K et al: Subependymoma of the cerebellopontine angle and prepontine cistern in a 15-year-old adolescent boy. AJNR Am J Neuroradiol. 29(1):190-1, 2008
                                                                                          22. Romoli S et al: Unusual exophytic subependymoma in the bulbo-cerebellar angle. Case report. J Neurosurg Sci. 51(2):81-4, 2007
                                                                                          23. Rushing EJ et al: Subependymoma revisited: clinicopathological evaluation of 83 cases. J Neurooncol. 85(3):297-305, 2007
                                                                                          24. Ragel BT et al: Subependymomas: an analysis of clinical and imaging features. Neurosurgery. 58(5):881-90; discussion 881-90, 2006
                                                                                          25. Rath TJ et al: Massive symptomatic subependymoma of the lateral ventricles: case report and review of the literature. Neuroradiology. 47(3):183-8, 2005
                                                                                          26. Shuangshoti S et al: Supratentorial extraventricular ependymal neoplasms: a clinicopathologic study of 32 patients. Cancer. 103(12):2598-605, 2005
                                                                                          27. Kim HC et al: Subependymoma in the third ventricle in a child. Clin Imaging. 28(5):381-4, 2004
                                                                                          28. Im SH et al: Clinicopathological study of seven cases of symptomatic supratentorial subependymoma. J Neurooncol. 61(1):57-67, 2003
                                                                                          29. Seol HJ et al: A case of recurrent subependymoma with subependymal seeding: case report. J Neurooncol. 62(3):315-20, 2003
                                                                                          30. Burger PC et al: Surgical pathology of the nervous system and its coverings: The Brain: Tumors. 4th ed. Philadelphia: Churchill Livingstone. 250-4, 2002
                                                                                          31. Ironside JW et al: Diagnostic pathology of nervous system tumours: Ependymal and choroid plexus tumors. 1st ed. Edinburgh: Churchill Livingstone. 145-83, 2002
                                                                                          32. Nishio S et al: Tumours around the foramen of Monro: clinical and neuroimaging features and their differential diagnosis. J Clin Neurosci. 9(2):137-41, 2002
                                                                                          33. Fontenele GI et al: Symptomatic child case of subependymoma in the fourth ventricle without hydrocephalus. Radiat Med. 19(1):37-42, 2001
                                                                                          34. Wiestler OD et al: Pathology and genetics of tumours of the nervous system: Subependymoma. Lyon: IARC Press. 80-1, 2000
                                                                                          35. Maiuri F et al: Symptomatic subependymomas of the lateral ventricles. Report of eight cases. Clin Neurol Neurosurg. 99(1):17-22, 1997
                                                                                          36. Mineura K et al: Subependymoma of the septum pellucidum: characterization by PET. J Neurooncol. 32(2):143-7, 1997
                                                                                          37. Jallo GI et al: Intramedullary subependymoma of the spinal cord. Neurosurgery. 38(2):251-7, 1996
                                                                                          38. Chiechi MV et al: Intracranial subependymomas: CT and MR imaging features in 24 cases. AJR Am J Roentgenol. 165(5):1245-50, 1995
                                                                                          39. Furie DM et al: Supratentorial ependymomas and subependymomas: CT and MR appearance. J Comput Assist Tomogr. 19(4):518-26, 1995
                                                                                          40. Hoeffel C et al: MR manifestations of subependymomas. AJNR Am J Neuroradiol. 16(10):2121-9, 1995
                                                                                          41. Silverstein JE et al: MRI of intracranial subependymomas. J Comput Assist Tomogr. 19(2):264-7, 1995
                                                                                          42. Iqbal Z et al: Subependymoma of the lateral ventricle: case report and literature review. Br J Neurosurg. 8(1):83-5, 1994
                                                                                          43. Ryken TC et al: Familial occurrence of subependymoma. Report of two cases. J Neurosurg. 80(6):1108-11, 1994
                                                                                          44. Lindboe CF et al: Hemorrhage in a highly vascularized subependymoma of the septum pellucidum: case report. Neurosurgery. 31(4):741-5, 1992
                                                                                          45. Spoto GP et al: Intracranial ependymoma and subependymoma: MR manifestations. AJNR Am J Neuroradiol. 11(1):83-91, 1990