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Subependymal glioma

MedGen UID:
64637
•Concept ID:
C0206725
•
Neoplastic Process
Synonym: Subependymoma
SNOMED CT: Mixed subependymoma-ependymoma (4553004); Subependymal glioma (4553004); Subependymoma (4553004); Subependymal astrocytoma (4553004)
 
Monarch Initiative: MONDO:0007667
OMIM®: 137800
Orphanet: ORPHA251639

Definition

Subependymoma is a rare and slow growing type of ependymoma (see this term), often presenting in middle-aged adults, found more commonly in men than in women, usually located in the fourth and lateral ventricles and manifesting with variable symptoms including headache, nausea, and loss of balance. In some cases it can be asymptomatic. It is usually associated with a better prognosis than other forms of ependymoma. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
Follow this link to review classifications for Subependymal glioma in Orphanet.

Professional guidelines

PubMed

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
Biology and management of ependymomas.
Wu J, Armstrong TS, Gilbert MR
Neuro Oncol 2016 Jul;18(7):902-13. Epub 2016 Mar 28 doi: 10.1093/neuonc/now016. PMID: 27022130Free PMC Article
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

Subependymomas of the fourth ventricle: To operate or not to operate?
Haider AS, McCutcheon IE, Ene CI, Fuller GN, Schomer DF, Gule-Monroe M, DeMonte F, Ferguson SD, Lang FF, Prabhu SS, Raza SM, Suki D, Weinberg JS, Sawaya R
J Clin Neurosci 2023 Dec;118:147-152. Epub 2023 Nov 7 doi: 10.1016/j.jocn.2023.10.025. PMID: 37944358
Survival of patients and risk factors for subependymoma: a population-based study.
Zhang GJ, Cheng X, Chen C, You C
Neurol Res 2023 Feb;45(2):173-180. Epub 2022 Sep 25 doi: 10.1080/01616412.2022.2127250. PMID: 36153833
Imaging characteristics of 4th ventricle subependymoma.
Haider AS, El Ahmadieh TY, Haider M, Hatanpaa KJ, Pinho MC, Mickey BE, Sawaya R, Fuller GN, Schomer DF, Gule-Monroe M
Neuroradiology 2022 Sep;64(9):1795-1800. Epub 2022 Apr 15 doi: 10.1007/s00234-022-02944-7. PMID: 35426054Free PMC Article
Tumor characteristics and surgical outcomes of intracranial subependymomas: a systematic review and meta-analysis.
Kweh BTS, Rosenfeld JV, Hunn M, Tee JW
J Neurosurg 2022 Mar 1;136(3):736-748. Epub 2021 Aug 20 doi: 10.3171/2021.2.JNS204052. PMID: 34416731
Biology and management of ependymomas.
Wu J, Armstrong TS, Gilbert MR
Neuro Oncol 2016 Jul;18(7):902-13. Epub 2016 Mar 28 doi: 10.1093/neuonc/now016. PMID: 27022130Free PMC Article

Diagnosis

Tumors of Choroid Plexus and Other Ventricular Tumors.
Spennato P, De Martino L, Russo C, Errico ME, Imperato A, Mazio F, Miccoli G, Quaglietta L, Abate M, Covelli E, Donofrio V, Cinalli G
Adv Exp Med Biol 2023;1405:175-223. doi: 10.1007/978-3-031-23705-8_7. PMID: 37452939
Imaging characteristics of 4th ventricle subependymoma.
Haider AS, El Ahmadieh TY, Haider M, Hatanpaa KJ, Pinho MC, Mickey BE, Sawaya R, Fuller GN, Schomer DF, Gule-Monroe M
Neuroradiology 2022 Sep;64(9):1795-1800. Epub 2022 Apr 15 doi: 10.1007/s00234-022-02944-7. PMID: 35426054Free PMC Article
Surgical management and long-term outcome of intracranial subependymoma.
Varma A, Giraldi D, Mills S, Brodbelt AR, Jenkinson MD
Acta Neurochir (Wien) 2018 Sep;160(9):1793-1799. Epub 2018 Jun 18 doi: 10.1007/s00701-018-3570-4. PMID: 29915887Free PMC Article
Biology and management of ependymomas.
Wu J, Armstrong TS, Gilbert MR
Neuro Oncol 2016 Jul;18(7):902-13. Epub 2016 Mar 28 doi: 10.1093/neuonc/now016. PMID: 27022130Free PMC Article
Ependymomas.
Chamberlain MC
Curr Neurol Neurosci Rep 2003 May;3(3):193-9. doi: 10.1007/s11910-003-0078-x. PMID: 12691623

Therapy

Spinal subependymoma surgery: do no harm. Little may be more!
Soleiman HA, Ironside J, Kealey S, Demetriades AK
Neurosurg Rev 2020 Aug;43(4):1047-1053. Epub 2019 Jun 18 doi: 10.1007/s10143-019-01128-x. PMID: 31214945
Surgical management and long-term outcome of intracranial subependymoma.
Varma A, Giraldi D, Mills S, Brodbelt AR, Jenkinson MD
Acta Neurochir (Wien) 2018 Sep;160(9):1793-1799. Epub 2018 Jun 18 doi: 10.1007/s00701-018-3570-4. PMID: 29915887Free PMC Article
Differentiating the mTOR inhibitors everolimus and sirolimus in the treatment of tuberous sclerosis complex.
MacKeigan JP, Krueger DA
Neuro Oncol 2015 Dec;17(12):1550-9. Epub 2015 Aug 19 doi: 10.1093/neuonc/nov152. PMID: 26289591Free PMC Article
Therapeutic targets in subependymoma.
Kong LY, Wei J, Haider AS, Liebelt BD, Ling X, Conrad CA, Fuller GN, Levine NB, Priebe W, Sawaya R, Heimberger AB
J Neuroimmunol 2014 Dec 15;277(1-2):168-75. Epub 2014 Oct 31 doi: 10.1016/j.jneuroim.2014.10.008. PMID: 25465288
Ependymomas.
Chamberlain MC
Curr Neurol Neurosci Rep 2003 May;3(3):193-9. doi: 10.1007/s11910-003-0078-x. PMID: 12691623

Prognosis

Survival of patients and risk factors for subependymoma: a population-based study.
Zhang GJ, Cheng X, Chen C, You C
Neurol Res 2023 Feb;45(2):173-180. Epub 2022 Sep 25 doi: 10.1080/01616412.2022.2127250. PMID: 36153833
Tumor characteristics and surgical outcomes of intracranial subependymomas: a systematic review and meta-analysis.
Kweh BTS, Rosenfeld JV, Hunn M, Tee JW
J Neurosurg 2022 Mar 1;136(3):736-748. Epub 2021 Aug 20 doi: 10.3171/2021.2.JNS204052. PMID: 34416731
Intracranial Subependymoma: A SEER Analysis 2004-2013.
Nguyen HS, Doan N, Gelsomino M, Shabani S
World Neurosurg 2017 May;101:599-605. Epub 2017 Feb 15 doi: 10.1016/j.wneu.2017.02.019. PMID: 28232153
Ependymomas.
Chamberlain MC
Curr Neurol Neurosci Rep 2003 May;3(3):193-9. doi: 10.1007/s11910-003-0078-x. PMID: 12691623
Symptomatic subependymomas of the lateral ventricles. Report of eight cases.
Maiuri F, Gangemi M, Iaconetta G, Signorelli F, Del Basso De Caro M
Clin Neurol Neurosurg 1997 Feb;99(1):17-22. doi: 10.1016/s0303-8467(96)00554-9. PMID: 9107462

Clinical prediction guides

Subependymomas of the fourth ventricle: To operate or not to operate?
Haider AS, McCutcheon IE, Ene CI, Fuller GN, Schomer DF, Gule-Monroe M, DeMonte F, Ferguson SD, Lang FF, Prabhu SS, Raza SM, Suki D, Weinberg JS, Sawaya R
J Clin Neurosci 2023 Dec;118:147-152. Epub 2023 Nov 7 doi: 10.1016/j.jocn.2023.10.025. PMID: 37944358
Intramedullary Subependymoma Of Lower Thoracic Cord.
Bashir HH, Aziz HF, Shamim MS
J Pak Med Assoc 2023 Sep;73(9):1919-1920. doi: 10.47391/JPMA.23-66. PMID: 37817716
Tumor characteristics and surgical outcomes of intracranial subependymomas: a systematic review and meta-analysis.
Kweh BTS, Rosenfeld JV, Hunn M, Tee JW
J Neurosurg 2022 Mar 1;136(3):736-748. Epub 2021 Aug 20 doi: 10.3171/2021.2.JNS204052. PMID: 34416731
Surgical management and long-term outcome of intracranial subependymoma.
Varma A, Giraldi D, Mills S, Brodbelt AR, Jenkinson MD
Acta Neurochir (Wien) 2018 Sep;160(9):1793-1799. Epub 2018 Jun 18 doi: 10.1007/s00701-018-3570-4. PMID: 29915887Free PMC Article
Therapeutic targets in subependymoma.
Kong LY, Wei J, Haider AS, Liebelt BD, Ling X, Conrad CA, Fuller GN, Levine NB, Priebe W, Sawaya R, Heimberger AB
J Neuroimmunol 2014 Dec 15;277(1-2):168-75. Epub 2014 Oct 31 doi: 10.1016/j.jneuroim.2014.10.008. PMID: 25465288

Recent systematic reviews

Tumor characteristics and surgical outcomes of intracranial subependymomas: a systematic review and meta-analysis.
Kweh BTS, Rosenfeld JV, Hunn M, Tee JW
J Neurosurg 2022 Mar 1;136(3):736-748. Epub 2021 Aug 20 doi: 10.3171/2021.2.JNS204052. PMID: 34416731

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