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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
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
Advances in the management of subependymal giant cell astrocytoma.
Beaumont TL, Limbrick DD, Smyth MD
Childs Nerv Syst 2012 Jul;28(7):963-8. Epub 2012 May 5 doi: 10.1007/s00381-012-1785-x. PMID: 22562196

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
Subependymal giant-cell astrocytoma: A surgical review in the modern era of mTOR inhibitors.
Danassegarane G, Tinois J, Sahler Y, Aouaissia S, Riffaud L
Neurochirurgie 2022 Dec;68(6):627-636. Epub 2022 Jul 27 doi: 10.1016/j.neuchi.2022.07.003. PMID: 35907444
Neurosurgical Considerations of Neurocutaneous Syndromes.
Iyer RR, Strahle JM, Groves ML
Neurosurg Clin N Am 2022 Jan;33(1):81-89. Epub 2021 Oct 28 doi: 10.1016/j.nec.2021.09.013. PMID: 34801145
Neurological malignancies in neurofibromatosis type 1.
Eoli M, Saletti V, Finocchiaro G
Curr Opin Oncol 2019 Nov;31(6):554-561. doi: 10.1097/CCO.0000000000000576. PMID: 31436563
Everolimus.
Hasskarl J
Recent Results Cancer Res 2018;211:101-123. doi: 10.1007/978-3-319-91442-8_8. PMID: 30069763

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
Familial Nervous System Tumor Syndromes.
Strowd RE 3rd, Plotkin SR
Continuum (Minneap Minn) 2020 Dec;26(6):1523-1552. doi: 10.1212/CON.0000000000000950. PMID: 33273171
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
Tuberous sclerosis complex.
Islam MP, Roach ES
Handb Clin Neurol 2015;132:97-109. doi: 10.1016/B978-0-444-62702-5.00006-8. PMID: 26564073
Tuberous sclerosis complex.
DiMario FJ Jr, Sahin M, Ebrahimi-Fakhari D
Pediatr Clin North Am 2015 Jun;62(3):633-48. Epub 2015 Apr 11 doi: 10.1016/j.pcl.2015.03.005. PMID: 26022167

Therapy

Mechanistic Target of Rapamycin (mTOR) Inhibitors.
Wang D, Eisen HJ
Handb Exp Pharmacol 2022;272:53-72. doi: 10.1007/164_2021_553. PMID: 35091825
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
Everolimus.
Hasskarl J
Recent Results Cancer Res 2014;201:373-92. doi: 10.1007/978-3-642-54490-3_23. PMID: 24756805
Advances in the management of subependymal giant cell astrocytoma.
Beaumont TL, Limbrick DD, Smyth MD
Childs Nerv Syst 2012 Jul;28(7):963-8. Epub 2012 May 5 doi: 10.1007/s00381-012-1785-x. PMID: 22562196
Everolimus tablets for patients with subependymal giant cell astrocytoma.
Turner SG, Peters KB, Vredenburgh JJ, Desjardins A, Friedman HS, Reardon DA
Expert Opin Pharmacother 2011 Oct;12(14):2265-9. Epub 2011 Aug 1 doi: 10.1517/14656566.2011.601742. PMID: 21806479Free PMC Article

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
Neurological malignancies in neurofibromatosis type 1.
Eoli M, Saletti V, Finocchiaro G
Curr Opin Oncol 2019 Nov;31(6):554-561. doi: 10.1097/CCO.0000000000000576. PMID: 31436563
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
Perinatal (fetal and neonatal) astrocytoma: a review.
Isaacs H Jr
Childs Nerv Syst 2016 Nov;32(11):2085-2096. Epub 2016 Aug 27 doi: 10.1007/s00381-016-3215-y. PMID: 27568373Free PMC Article
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

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
Infantile atypical subependymal giant cell astrocytoma.
Almubarak AO, Abdullah J, Al Hindi H, AlShail E
Neurosciences (Riyadh) 2020 Jan;25(1):61-64. doi: 10.17712/nsj.2020.1.20190044. PMID: 31982898Free PMC Article
Neurological malignancies in neurofibromatosis type 1.
Eoli M, Saletti V, Finocchiaro G
Curr Opin Oncol 2019 Nov;31(6):554-561. doi: 10.1097/CCO.0000000000000576. PMID: 31436563
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

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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