U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

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.

Professional guidelines

PubMed

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
Wirsching HG, Weller M
Curr Treat Options Oncol 2016 Oct;17(10):51. doi: 10.1007/s11864-016-0430-4. PMID: 27501915
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

Therapy

Cockerell I, Christensen J, Hoei-Hansen CE, Holst L, Grenaa Frederiksen M, Issa-Epe AI, Nedregaard B, Solhoff R, Heimdal K, Johannessen Landmark C, Lund C, Nærland T
Orphanet J Rare Dis 2023 Dec 2;18(1):377. doi: 10.1186/s13023-023-02982-1. PMID: 38042867Free PMC Article
Wang D, Eisen HJ
Handb Exp Pharmacol 2022;272:53-72. doi: 10.1007/164_2021_553. PMID: 35091825
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
De Waele L, Lagae L, Mekahli D
Pediatr Nephrol 2015 Oct;30(10):1771-80. Epub 2014 Dec 23 doi: 10.1007/s00467-014-3027-9. PMID: 25533384
Hasskarl J
Recent Results Cancer Res 2014;201:373-92. doi: 10.1007/978-3-642-54490-3_23. PMID: 24756805

Prognosis

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
Eoli M, Saletti V, Finocchiaro G
Curr Opin Oncol 2019 Nov;31(6):554-561. doi: 10.1097/CCO.0000000000000576. PMID: 31436563
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
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
De Waele L, Lagae L, Mekahli D
Pediatr Nephrol 2015 Oct;30(10):1771-80. Epub 2014 Dec 23 doi: 10.1007/s00467-014-3027-9. PMID: 25533384

Clinical prediction guides

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
Eoli M, Saletti V, Finocchiaro G
Curr Opin Oncol 2019 Nov;31(6):554-561. doi: 10.1097/CCO.0000000000000576. PMID: 31436563
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
Wirsching HG, Weller M
Curr Treat Options Oncol 2016 Oct;17(10):51. doi: 10.1007/s11864-016-0430-4. PMID: 27501915
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

Piña-Ballantyne SA, Espinosa-Aguilar EJ, Calderón-Garcidueñas AL
Neurol India 2024 Jul 1;72(4):708-717. Epub 2024 Aug 31 doi: 10.4103/neurol-india.Neurol-India-D-23-00343. PMID: 39216021
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
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
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
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

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...