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
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
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
Beaumont TL et al: Subependymal giant cell astrocytoma in the absence of tuberous sclerosis complex: case report. J Neurosurg Pediatr. 1-4, 2015
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
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
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
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
Kotulska K et al: Surgical treatment of subependymal giant cell astrocytoma in tuberous sclerosis complex patients. Pediatr Neurol. 50(4):307-12, 2014
Ouyang T et al: Subependymal giant cell astrocytoma: current concepts, management, and future directions. Childs Nerv Syst. 30(4):561-70, 2014
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
Krueger DA et al: Everolimus long-term safety and efficacy in subependymal giant cell astrocytoma. Neurology. 80(6):574-80, 2013
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
Buccoliero AM et al: Subependymal giant cell astrocytoma (SEGA): is it an astrocytoma? Morphological, immunohistochemical and ultrastructural study. Neuropathology. 29(1):25-30, 2009
Ess KC et al: Developmental origin of subependymal giant cell astrocytoma in tuberous sclerosis complex. Neurology. 64(8):1446-9, 2005
You H et al: Immunohistochemical study of central neurocytoma, subependymoma, and subependymal giant cell astrocytoma. J Neurooncol. 74(1):1-8, 2005
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
Cuccia V et al: Subependymal giant cell astrocytoma in children with tuberous sclerosis. Childs Nerv Syst. 19(4):232-43, 2003
Koeller KK et al: From the archives of the AFIP. Cerebral intraventricular neoplasms: radiologic-pathologic correlation. Radiographics. 22(6):1473-505, 2002
Medhkour A et al: Neonatal subependymal giant cell astrocytoma. Pediatr Neurosurg. 36(5):271-4, 2002
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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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
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
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
Beaumont TL et al: Subependymal giant cell astrocytoma in the absence of tuberous sclerosis complex: case report. J Neurosurg Pediatr. 1-4, 2015
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
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
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
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
Kotulska K et al: Surgical treatment of subependymal giant cell astrocytoma in tuberous sclerosis complex patients. Pediatr Neurol. 50(4):307-12, 2014
Ouyang T et al: Subependymal giant cell astrocytoma: current concepts, management, and future directions. Childs Nerv Syst. 30(4):561-70, 2014
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
Krueger DA et al: Everolimus long-term safety and efficacy in subependymal giant cell astrocytoma. Neurology. 80(6):574-80, 2013
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
Buccoliero AM et al: Subependymal giant cell astrocytoma (SEGA): is it an astrocytoma? Morphological, immunohistochemical and ultrastructural study. Neuropathology. 29(1):25-30, 2009
Ess KC et al: Developmental origin of subependymal giant cell astrocytoma in tuberous sclerosis complex. Neurology. 64(8):1446-9, 2005
You H et al: Immunohistochemical study of central neurocytoma, subependymoma, and subependymal giant cell astrocytoma. J Neurooncol. 74(1):1-8, 2005
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
Cuccia V et al: Subependymal giant cell astrocytoma in children with tuberous sclerosis. Childs Nerv Syst. 19(4):232-43, 2003
Koeller KK et al: From the archives of the AFIP. Cerebral intraventricular neoplasms: radiologic-pathologic correlation. Radiographics. 22(6):1473-505, 2002
Medhkour A et al: Neonatal subependymal giant cell astrocytoma. Pediatr Neurosurg. 36(5):271-4, 2002
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
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