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Subependymal Giant Cell Astrocytoma
Luke L. Linscott, MD; Anne G. Osborn, MD, FACR; John H. Rees, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Subependymal giant cell astrocytoma (SGCA, SEGA)
              • Synonyms

                • Intraventricular astrocytoma of tuberous sclerosis complex (TSC)
              • Definitions

                • Benign, slow-growing glioneuronal tumor usually arising adjacent to foramen of Monro in TSC

              IMAGING

              • General Features

                • CT Findings

                  • MR Findings

                    • Ultrasonographic Findings

                      • Angiographic 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. Trelinska J et al: Maintenance therapy with everolimus for subependymal giant cell astrocytoma in patients with tuberous sclerosis (the EMINENTS study). Pediatr Blood Cancer. 64(6), 2017
                                                2. French JA et al: Adjunctive everolimus therapy for treatment-resistant focal-onset seizures associated with tuberous sclerosis (EXIST-3): a phase 3, randomised, double-blind, placebo-controlled study. Lancet. 388(10056):2153-63, 2016
                                                3. Beaumont TL et al: Subependymal giant cell astrocytoma in the absence of tuberous sclerosis complex: case report. J Neurosurg Pediatr. 1-4, 2015
                                                4. Kwiatkowski DJ et al: Response to everolimus is seen in TSC-associated SEGAs and angiomyolipomas independent of mutation type and site in TSC1 and TSC2. Eur J Hum Genet. 23(12):1665-72, 2015
                                                5. Cardamone M et al: Mammalian target of rapamycin inhibitors for intractable epilepsy and subependymal giant cell astrocytomas in tuberous sclerosis complex. J Pediatr. 164(5):1195-200, 2014
                                                6. Franz DN et al: Everolimus for subependymal giant cell astrocytoma in patients with tuberous sclerosis complex: 2-year open-label extension of the randomised EXIST-1 study. Lancet Oncol. 15(13):1513-20, 2014
                                                7. Harter DH et al: A management strategy for intraventricular subependymal giant cell astrocytomas in tuberous sclerosis complex. J Neurosurg Pediatr. 13(1):21-8, 2014
                                                8. Kotulska K et al: Surgical treatment of subependymal giant cell astrocytoma in tuberous sclerosis complex patients. Pediatr Neurol. 50(4):307-12, 2014
                                                9. Ouyang T et al: Subependymal giant cell astrocytoma: current concepts, management, and future directions. Childs Nerv Syst. 30(4):561-70, 2014
                                                10. Rovira À et al: Recommendations for the radiological diagnosis and follow-up of neuropathological abnormalities associated with tuberous sclerosis complex. J Neurooncol. 118(2):205-23, 2014
                                                11. Krueger DA et al: Everolimus long-term safety and efficacy in subependymal giant cell astrocytoma. Neurology. 80(6):574-80, 2013
                                                12. Roth J et al: Subependymal giant cell astrocytoma: diagnosis, screening, and treatment. Recommendations from the International Tuberous Sclerosis Complex Consensus Conference 2012. Pediatr Neurol. 49(6):439-44, 2013
                                                13. Buccoliero AM et al: Subependymal giant cell astrocytoma (SEGA): is it an astrocytoma? Morphological, immunohistochemical and ultrastructural study. Neuropathology. 29(1):25-30, 2009
                                                14. Ess KC et al: Developmental origin of subependymal giant cell astrocytoma in tuberous sclerosis complex. Neurology. 64(8):1446-9, 2005
                                                15. You H et al: Immunohistochemical study of central neurocytoma, subependymoma, and subependymal giant cell astrocytoma. J Neurooncol. 74(1):1-8, 2005
                                                16. Chan JA et al: Pathogenesis of tuberous sclerosis subependymal giant cell astrocytomas: biallelic inactivation of TSC1 or TSC2 leads to mTOR activation. J Neuropathol Exp Neurol. 63(12):1236-42, 2004
                                                17. Cuccia V et al: Subependymal giant cell astrocytoma in children with tuberous sclerosis. Childs Nerv Syst. 19(4):232-43, 2003
                                                18. Koeller KK et al: From the archives of the AFIP. Cerebral intraventricular neoplasms: radiologic-pathologic correlation. Radiographics. 22(6):1473-505, 2002
                                                19. Medhkour A et al: Neonatal subependymal giant cell astrocytoma. Pediatr Neurosurg. 36(5):271-4, 2002
                                                20. 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
                                                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

                                                            • Abbreviations

                                                              • Subependymal giant cell astrocytoma (SGCA, SEGA)
                                                            • Synonyms

                                                              • Intraventricular astrocytoma of tuberous sclerosis complex (TSC)
                                                            • Definitions

                                                              • Benign, slow-growing glioneuronal tumor usually arising adjacent to foramen of Monro in TSC

                                                            IMAGING

                                                            • General Features

                                                              • CT Findings

                                                                • MR Findings

                                                                  • Ultrasonographic Findings

                                                                    • Angiographic 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. Trelinska J et al: Maintenance therapy with everolimus for subependymal giant cell astrocytoma in patients with tuberous sclerosis (the EMINENTS study). Pediatr Blood Cancer. 64(6), 2017
                                                                                              2. French JA et al: Adjunctive everolimus therapy for treatment-resistant focal-onset seizures associated with tuberous sclerosis (EXIST-3): a phase 3, randomised, double-blind, placebo-controlled study. Lancet. 388(10056):2153-63, 2016
                                                                                              3. Beaumont TL et al: Subependymal giant cell astrocytoma in the absence of tuberous sclerosis complex: case report. J Neurosurg Pediatr. 1-4, 2015
                                                                                              4. Kwiatkowski DJ et al: Response to everolimus is seen in TSC-associated SEGAs and angiomyolipomas independent of mutation type and site in TSC1 and TSC2. Eur J Hum Genet. 23(12):1665-72, 2015
                                                                                              5. Cardamone M et al: Mammalian target of rapamycin inhibitors for intractable epilepsy and subependymal giant cell astrocytomas in tuberous sclerosis complex. J Pediatr. 164(5):1195-200, 2014
                                                                                              6. Franz DN et al: Everolimus for subependymal giant cell astrocytoma in patients with tuberous sclerosis complex: 2-year open-label extension of the randomised EXIST-1 study. Lancet Oncol. 15(13):1513-20, 2014
                                                                                              7. Harter DH et al: A management strategy for intraventricular subependymal giant cell astrocytomas in tuberous sclerosis complex. J Neurosurg Pediatr. 13(1):21-8, 2014
                                                                                              8. Kotulska K et al: Surgical treatment of subependymal giant cell astrocytoma in tuberous sclerosis complex patients. Pediatr Neurol. 50(4):307-12, 2014
                                                                                              9. Ouyang T et al: Subependymal giant cell astrocytoma: current concepts, management, and future directions. Childs Nerv Syst. 30(4):561-70, 2014
                                                                                              10. Rovira À et al: Recommendations for the radiological diagnosis and follow-up of neuropathological abnormalities associated with tuberous sclerosis complex. J Neurooncol. 118(2):205-23, 2014
                                                                                              11. Krueger DA et al: Everolimus long-term safety and efficacy in subependymal giant cell astrocytoma. Neurology. 80(6):574-80, 2013
                                                                                              12. Roth J et al: Subependymal giant cell astrocytoma: diagnosis, screening, and treatment. Recommendations from the International Tuberous Sclerosis Complex Consensus Conference 2012. Pediatr Neurol. 49(6):439-44, 2013
                                                                                              13. Buccoliero AM et al: Subependymal giant cell astrocytoma (SEGA): is it an astrocytoma? Morphological, immunohistochemical and ultrastructural study. Neuropathology. 29(1):25-30, 2009
                                                                                              14. Ess KC et al: Developmental origin of subependymal giant cell astrocytoma in tuberous sclerosis complex. Neurology. 64(8):1446-9, 2005
                                                                                              15. You H et al: Immunohistochemical study of central neurocytoma, subependymoma, and subependymal giant cell astrocytoma. J Neurooncol. 74(1):1-8, 2005
                                                                                              16. Chan JA et al: Pathogenesis of tuberous sclerosis subependymal giant cell astrocytomas: biallelic inactivation of TSC1 or TSC2 leads to mTOR activation. J Neuropathol Exp Neurol. 63(12):1236-42, 2004
                                                                                              17. Cuccia V et al: Subependymal giant cell astrocytoma in children with tuberous sclerosis. Childs Nerv Syst. 19(4):232-43, 2003
                                                                                              18. Koeller KK et al: From the archives of the AFIP. Cerebral intraventricular neoplasms: radiologic-pathologic correlation. Radiographics. 22(6):1473-505, 2002
                                                                                              19. Medhkour A et al: Neonatal subependymal giant cell astrocytoma. Pediatr Neurosurg. 36(5):271-4, 2002
                                                                                              20. 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