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Subependymal giant-cell astrocytoma(SEGA)

MedGen UID:
61446
•Concept ID:
C0205768
•
Neoplastic Process
Synonym: SEGA
SNOMED CT: SEGA - Subependymal giant cell astrocytoma (449799008); Subependymal giant cell astrocytoma (449799008); Subependymal giant cell astrocytoma (1586004)
 
HPO: HP:0009718
Monarch Initiative: MONDO:0016693
Orphanet: ORPHA251618

Definition

A demarcated, largely intraventricular tumor in the region of the foramen of Monro composed of spindle to large plump or ganglion-like cells with eosinophilic to amphophilic cytoplasm and somewhat pleomorphic nuclei with occasional prominent nucleoli. These tumors are almost always associated with tuberous sclerosis. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • Subependymal giant-cell astrocytoma
Follow this link to review classifications for Subependymal giant-cell astrocytoma in Orphanet.

Conditions with this feature

Tuberous sclerosis 2
MedGen UID:
348170
•Concept ID:
C1860707
•
Disease or Syndrome
Tuberous sclerosis complex (TSC) involves abnormalities of the skin (hypomelanotic macules, confetti skin lesions, facial angiofibromas, shagreen patches, fibrous cephalic plaques, ungual fibromas); brain (subependymal nodules, cortical tubers, and subependymal giant cell astrocytomas [SEGAs], seizures, intellectual disability / developmental delay, psychiatric illness); kidney (angiomyolipomas, cysts, renal cell carcinomas); heart (rhabdomyomas, arrhythmias); and lungs (lymphangioleiomyomatosis [LAM], multifocal micronodular pneumonocyte hyperplasia). Central nervous system tumors are the leading cause of morbidity and mortality; renal disease is the second leading cause of early death.
See: Condition Record
Tuberous sclerosis 2

Professional guidelines

PubMed

Pharmacological treatment strategies for subependymal giant cell astrocytoma (SEGA).
Ebrahimi-Fakhari D, Franz DN
Expert Opin Pharmacother 2020 Aug;21(11):1329-1336. Epub 2020 Apr 27 doi: 10.1080/14656566.2020.1751124. PMID: 32338549
The Role of Molecular Diagnostics in the Management of Patients with Gliomas.
Wirsching HG, Weller M
Curr Treat Options Oncol 2016 Oct;17(10):51. doi: 10.1007/s11864-016-0430-4. PMID: 27501915
Intraocular astrocytoma and its differential diagnosis.
Pusateri A, Margo CE
Arch Pathol Lab Med 2014 Sep;138(9):1250-4. doi: 10.5858/arpa.2013-0448-RS. PMID: 25171711

Recent clinical studies

Etiology

Benign Glioma.
Wu PB, Filley AC, Miller ML, Bruce JN
Adv Exp Med Biol 2023;1405:31-71. doi: 10.1007/978-3-031-23705-8_2. PMID: 37452934
Everolimus.
Hasskarl J
Recent Results Cancer Res 2018;211:101-123. doi: 10.1007/978-3-319-91442-8_8. PMID: 30069763
Characterization of gliomas: from morphology to molecules.
Ferris SP, Hofmann JW, Solomon DA, Perry A
Virchows Arch 2017 Aug;471(2):257-269. Epub 2017 Jul 4 doi: 10.1007/s00428-017-2181-4. PMID: 28674742
Direct medical costs for patients with tuberous sclerosis complex and surgical resection of subependymal giant cell astrocytoma: a US national cohort study.
Sun P, Liu Z, Krueger D, Kohrman M
J Med Econ 2015 May;18(5):349-56. Epub 2015 Feb 3 doi: 10.3111/13696998.2014.1001513. PMID: 25525770
Rash to the mTOR inhibitor everolimus: systematic review and meta-analysis.
Ramirez-Fort MK, Case EC, Rosen AC, Cerci FB, Wu S, Lacouture ME
Am J Clin Oncol 2014 Jun;37(3):266-71. doi: 10.1097/COC.0b013e318277d62f. PMID: 23241507

Diagnosis

Benign Glioma.
Wu PB, Filley AC, Miller ML, Bruce JN
Adv Exp Med Biol 2023;1405:31-71. doi: 10.1007/978-3-031-23705-8_2. PMID: 37452934
Characterization of gliomas: from morphology to molecules.
Ferris SP, Hofmann JW, Solomon DA, Perry A
Virchows Arch 2017 Aug;471(2):257-269. Epub 2017 Jul 4 doi: 10.1007/s00428-017-2181-4. PMID: 28674742
The Role of Molecular Diagnostics in the Management of Patients with Gliomas.
Wirsching HG, Weller M
Curr Treat Options Oncol 2016 Oct;17(10):51. doi: 10.1007/s11864-016-0430-4. PMID: 27501915
Rare glial tumors.
Soffietti R, Rudà R, Reardon D
Handb Clin Neurol 2016;134:399-415. doi: 10.1016/B978-0-12-802997-8.00024-4. PMID: 26948368
Imaging findings of intraventricular and ependymal lesions.
Vandesteen L, Drier A, Galanaud D, Clarençon F, Leclercq D, Karachi C, Dormont D
J Neuroradiol 2013 Oct;40(4):229-44. Epub 2013 Jul 22 doi: 10.1016/j.neurad.2013.06.004. PMID: 23886872

Therapy

Rapamycin and rapalogs for tuberous sclerosis complex.
Sasongko TH, Kademane K, Chai Soon Hou S, Jocelyn TXY, Zabidi-Hussin Z
Cochrane Database Syst Rev 2023 Jul 11;7(7):CD011272. doi: 10.1002/14651858.CD011272.pub3. PMID: 37432030Free PMC Article
Resection of brain lesions with a neuroendoscopic ultrasonic aspirator - a systematic literature review.
Ebel F, Greuter L, Guzman R, Soleman J
Neurosurg Rev 2022 Oct;45(5):3109-3118. Epub 2022 Jul 27 doi: 10.1007/s10143-022-01837-w. PMID: 35896917Free PMC Article
Efficacy and safety of mTOR inhibitors (rapamycin and its analogues) for tuberous sclerosis complex: a meta-analysis.
Li M, Zhou Y, Chen C, Yang T, Zhou S, Chen S, Wu Y, Cui Y
Orphanet J Rare Dis 2019 Feb 13;14(1):39. doi: 10.1186/s13023-019-1012-x. PMID: 30760308Free PMC Article
The Role of mTOR Inhibitors in the Treatment of Patients with Tuberous Sclerosis Complex: Evidence-based and Expert Opinions.
Curatolo P, Bjørnvold M, Dill PE, Ferreira JC, Feucht M, Hertzberg C, Jansen A, Jóźwiak S, Kingswood JC, Kotulska K, Macaya A, Moavero R, Nabbout R, Zonnenberg BA
Drugs 2016 Apr;76(5):551-65. doi: 10.1007/s40265-016-0552-9. PMID: 26927950
Rash to the mTOR inhibitor everolimus: systematic review and meta-analysis.
Ramirez-Fort MK, Case EC, Rosen AC, Cerci FB, Wu S, Lacouture ME
Am J Clin Oncol 2014 Jun;37(3):266-71. doi: 10.1097/COC.0b013e318277d62f. PMID: 23241507

Prognosis

Benign Glioma.
Wu PB, Filley AC, Miller ML, Bruce JN
Adv Exp Med Biol 2023;1405:31-71. doi: 10.1007/978-3-031-23705-8_2. PMID: 37452934
Characterization of gliomas: from morphology to molecules.
Ferris SP, Hofmann JW, Solomon DA, Perry A
Virchows Arch 2017 Aug;471(2):257-269. Epub 2017 Jul 4 doi: 10.1007/s00428-017-2181-4. PMID: 28674742
The Role of Molecular Diagnostics in the Management of Patients with Gliomas.
Wirsching HG, Weller M
Curr Treat Options Oncol 2016 Oct;17(10):51. doi: 10.1007/s11864-016-0430-4. PMID: 27501915
Rare glial tumors.
Soffietti R, Rudà R, Reardon D
Handb Clin Neurol 2016;134:399-415. doi: 10.1016/B978-0-12-802997-8.00024-4. PMID: 26948368
Rash to the mTOR inhibitor everolimus: systematic review and meta-analysis.
Ramirez-Fort MK, Case EC, Rosen AC, Cerci FB, Wu S, Lacouture ME
Am J Clin Oncol 2014 Jun;37(3):266-71. doi: 10.1097/COC.0b013e318277d62f. PMID: 23241507

Clinical prediction guides

Rapamycin and rapalogs for tuberous sclerosis complex.
Sasongko TH, Kademane K, Chai Soon Hou S, Jocelyn TXY, Zabidi-Hussin Z
Cochrane Database Syst Rev 2023 Jul 11;7(7):CD011272. doi: 10.1002/14651858.CD011272.pub3. PMID: 37432030Free PMC Article
Efficacy, Retention and Tolerability of Everolimus in Patients with Tuberous Sclerosis Complex: A Survey-Based Study on Patients' Perspectives.
Willems LM, Rosenow F, Schubert-Bast S, Kurlemann G, Zöllner JP, Bast T, Bertsche A, Bettendorf U, Ebrahimi-Fakhari D, Grau J, Hahn A, Hartmann H, Hertzberg C, Hornemann F, Immisch I, Jacobs J, Klein KM, Klotz KA, Kluger G, Knake S, Knuf M, Marquard K, Mayer T, Meyer S, Muhle H, Müller-Schlüter K, von Podewils F, Ruf S, Sauter M, Schäfer H, Schlump JU, Syrbe S, Thiels C, Trollmann R, Wiemer-Kruel A, Wilken B, Zukunft B, Strzelczyk A
CNS Drugs 2021 Oct;35(10):1107-1122. Epub 2021 Jul 17 doi: 10.1007/s40263-021-00839-4. PMID: 34275102Free PMC Article
Characterization of gliomas: from morphology to molecules.
Ferris SP, Hofmann JW, Solomon DA, Perry A
Virchows Arch 2017 Aug;471(2):257-269. Epub 2017 Jul 4 doi: 10.1007/s00428-017-2181-4. PMID: 28674742
A circuitry and biochemical basis for tuberous sclerosis symptoms: from epilepsy to neurocognitive deficits.
Feliciano DM, Lin TV, Hartman NW, Bartley CM, Kubera C, Hsieh L, Lafourcade C, O'Keefe RA, Bordey A
Int J Dev Neurosci 2013 Nov;31(7):667-78. Epub 2013 Feb 26 doi: 10.1016/j.ijdevneu.2013.02.008. PMID: 23485365Free PMC Article
Tuberous sclerosis and epilepsy: role of astrocytes.
Wong M, Crino PB
Glia 2012 Aug;60(8):1244-50. Epub 2012 Mar 21 doi: 10.1002/glia.22326. PMID: 22438024

Recent systematic reviews

Rapamycin and rapalogs for tuberous sclerosis complex.
Sasongko TH, Kademane K, Chai Soon Hou S, Jocelyn TXY, Zabidi-Hussin Z
Cochrane Database Syst Rev 2023 Jul 11;7(7):CD011272. doi: 10.1002/14651858.CD011272.pub3. PMID: 37432030Free PMC Article
Resection of brain lesions with a neuroendoscopic ultrasonic aspirator - a systematic literature review.
Ebel F, Greuter L, Guzman R, Soleman J
Neurosurg Rev 2022 Oct;45(5):3109-3118. Epub 2022 Jul 27 doi: 10.1007/s10143-022-01837-w. PMID: 35896917Free PMC Article
Prevalence of BRAFV600 in glioma and use of BRAF Inhibitors in patients with BRAFV600 mutation-positive glioma: systematic review.
Andrews LJ, Thornton ZA, Saincher SS, Yao IY, Dawson S, McGuinness LA, Jones HE, Jefferies S, Short SC, Cheng HY, McAleenan A, Higgins JPT, Kurian KM
Neuro Oncol 2022 Apr 1;24(4):528-540. doi: 10.1093/neuonc/noab247. PMID: 34718782Free PMC Article
Rapamycin and rapalogs for tuberous sclerosis complex.
Sasongko TH, Ismail NF, Zabidi-Hussin Z
Cochrane Database Syst Rev 2016 Jul 13;7(7):CD011272. doi: 10.1002/14651858.CD011272.pub2. PMID: 27409709Free PMC Article
Rash to the mTOR inhibitor everolimus: systematic review and meta-analysis.
Ramirez-Fort MK, Case EC, Rosen AC, Cerci FB, Wu S, Lacouture ME
Am J Clin Oncol 2014 Jun;37(3):266-71. doi: 10.1097/COC.0b013e318277d62f. PMID: 23241507

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