U.S. flag

An official website of the United States government

NCBI Bookshelf. A service of the National Library of Medicine, National Institutes of Health.

StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan-.

Cover of StatPearls

StatPearls [Internet].

Show details


; .

Author Information and Affiliations

Last Update: June 26, 2023.

Continuing Education Activity

Ependymomas are glial cell tumors that commonly arise in the lining cells of the ventricular system, and less commonly outside the central nervous system (CNS), or within the brain parenchyma. They are comprised of genetically distinct subgroups of tumors and affect children more commonly than adults. This activity reviews the pathophysiology and presentation of ependymoma and highlights the role of the interprofessional team in their management.


  • Explain the causes of ependymoma.
  • Describe the presentation of ependymoma.
  • Summarize the treatment options for ependymoma.
  • Outline the importance of improving care coordination among interprofessional team members to improve outcomes for patients affected by ependymoma.
Access free multiple choice questions on this topic.


Ependymomas are glial cell tumors that commonly arise in the lining cells of the ventricular system, and less commonly outside the central nervous system (CNS), or within the brain parenchyma. They are comprised of genetically distinct subgroups of tumors and affect children more commonly than adults.[1]


While ependymomas that arise from different regions of the CNS are histologically similar, their clinical course often varies. Histological classification alone has not provided consistent and reliable survival outcomes in retrospective studies, meaning that ependymomas with similar histological grades may follow a substantially different clinical course. Recent studies suggest that these tumors may be categorized based on different populations of progenitor cells, which would explain different clinical courses in tumors with the same histological grade.[2] Several genetic abnormalities have been found to correlate with ependymoma and comprise large genomic regions.[3] Some of these studies have demonstrated that ependymomas correlate with distinct oncogenic products and molecular subgroups which may correlate more accurately with clinical outcomes compared with histologic classification alone.[4]


Ependymomas affect all age groups, with a higher frequency in children.[1] It is the third most common brain tumor in pediatric patients.[3]

As per the Central Brain Tumor Registry of the United States (CBTRUS) Statistical Report for CNS tumors from the years 2011 to 2015, ependymal tumors represent 1.7% of all brain and CNS tumors, with a median age of 44 years.[5]

In childhood, brain tumors are the primary cause of death associated with solid tumors. The most common pediatric brain tumors being medulloblastoma, pilocytic astrocytoma, brainstem glioma, and ependymoma.[6]


Based on genetic mutations and variations, ependymomas are classified as follows:

Posterior fossa (PF):

  • PF-EPN-SE (subependymoma)
  • PF-EPN-B (group B)
  • PF-EPN-A (group A)

Supratentorial (ST):

  • ST-EPN-SE (subependymoma)
  • ST-EPN-YAP1 (YAP1 fusions)
  • ST-EPN-RELA (RELA fusions)

Spinal ependymomas:

  • SP-EPN-MPE (myxopapillary)
  • SP-EPN-SE (subependymoma)
  • SP-EPN


As with other CNS tumors, the TNM system of classification does not apply to ependymomas as they rarely spread outside of the CNS, and the WHO classification for CNS tumors is the current standard used to classify these ependymomas:[1][7]

  • Grade I, slow-growing tumors which are often considered benign, including subependymomas and myxopapillary ependymomas:
    • Subependymomas typically arise in the ventricular walls, most commonly in the fourth or lateral ventricles - they are histologically characterized by a hypocellular tissue presenting clusters of cells with a bland nucleus surrounded by a glial matrix.
    • Myxopapillary ependymomas most commonly arise in the cauda equina, filum terminale, or conus medullaris, and present histologically as pseudopapillary structures with mucin-rich microcysts, the cells are cuboidal and radially arranged surrounding a myxoid stroma.
  • Grade II are ependymomas presenting with high cellularity and papillary structures. Cells are arranged regularly and present a clear cytoplasm.
  • Grade III, are anaplastic ependymomas, presenting with abundant mitotic cells with pseudopalisading necrosis.

History and Physical

As ependymomas arise within different CNS compartments, their clinical presentation may vary from acute onset increased intracranial pressure causing nausea and vomiting, to a more chronic and insidious clinical course.[4]

Ependymoma is a histological diagnosis, and it most commonly presents in three major anatomical sites: supratentorial, spinal cord, and infratentorial.[8]

Ependymoma is commonly suspected in adult patients suffering prolonged clinical courses, who present a non-enhancing and well-demarcated intraventricular lesion on brain imaging, isodense in computed tomography, and isointense on T1 images on magnetic resonance imaging (MRI).[9]


Traditionally, ependymomas are classified using the World Health Organization (WHO) system to classify tumors of the nervous system. This histological grading commonly aids in predicting the clinical and biological behavior of central nervous tumors. In ependymoma, however, the molecular and genomic profiles may provide more accurate information than only using histological grade.[10]

Tumor grading using the WHO classification system for nervous system tumors is based on different histological grades, this classification by itself would often not provide a reliable estimate of clinical course in patients with ependymoma. With increasing information about molecular pathogenetic mechanisms in ependymomas, the 2016 classification for nervous system tumors from WHO now includes in their classification the presence or absence of specific genetic alterations, RELA fusion-positive or negative, for ependymomas.[10][11]

There are nine molecular subgroups described in ependymomas, which correlate with different anatomical locations, genetics, and demographic characteristics. These new findings offer the opportunity of potentially improving the classification, management, and prognostic information for ependymomas based on their molecular subgroup.[8]

No current molecular or immunohistochemical markers are in routine use in the diagnostic workup for patients with ependymoma.[8]

Treatment / Management

Ependymal tumors are rare, constituting 1.7% of all brain tumors, as reported in the most recent CBTRUS statistical report; this poses a challenge in defining the optimal management for these entities. Retrospective studies have described improved survival in patients undergoing resection with adjuvant radiation therapy. There is minimal and limited evidence supporting chemotherapy for adult ependymomas.[4]

No established guidelines use the molecular subgroups to guide the treatment of ependymoma. The current consensus, however, recommends that patients with PF-EPN-A positive ependymoma, who are older than 12 months of age, undergo maximal safe micro-neurosurgical removal in addition to local radiotherapy.[8]

For intracranial ependymomas, surgery is typically the mainstay treatment. Complete resection without residual disease has presented better clinical outcomes and overall survival than partial resection. As discussed previously, there is insufficient evidence to support the use of chemotherapy.[8]

Differential Diagnosis

The differential diagnosis for tumors in the posterior fossa includes astrocytoma, medulloblastoma, choroid plexus tumors.

For supratentorial tumors, the differential includes glial tumors, choroid plexus carcinoma or papilloma, and embryonal tumors.[10]


The extent of surgical resection for intracranial ependymomas has been the most reliable and consistent independent prognostic factor. Intracranial ependymomas show a low metastatic potential and predominantly present a locally invasive histological pattern. Ependymomas that fall within the histological grade III from the WHO classification have correlated with a worse outcome than grade II.[12][13]

Patients who undergo complete resection without signs of residual disease have presented a better outcome and overall survival compared to patients who undergo partial resection. For this reason, treatment typically consists of aggressive surgical excision.[8]

Across the different subgroups, infratentorial ependymomas generally have an excellent prognosis, even without treatment. Supratentorial ependymomas, however, often present a higher histological grade and have a lower survival rate despite treatment with resection and adjuvant radiation.[4]

Prognosis varies for pediatric patients and will be subject to location, type of treatment, and pathological classification. A previous study showed that gross total resection had the best overall and progression-free survival. Patients with WHO classification grade II had improved overall survival after gross total resection in addition to external beam radiation therapy, and improved progression-free survival after gross total resection alone. Patients with WHO classification grade III had improved overall survival after subtotal resection in addition to external beam radiation therapy. While progression-free survival was better in patients with infratentorial tumors following subtotal resection in addition to external beam radiation therapy.[14][15][16][17]


Patients who are long-term survivors of CNS tumors may present a wide diversity of complications, including neurological deficits, cognitive limitations, sensorineural hearing loss, endocrine and growth abnormalities, and secondary malignancies. Adult patients may present long-term complications, most commonly fatigue, numbness and tingling, pain, and disturbed sleep.[15][16][18][19]

Deterrence and Patient Education

Patients should receive counseling regarding their prognosis. Those with CNS tumors at end-of-life benefit from palliative care. Family members and patients will benefit from early and anticipatory counseling, in addition to comfort measures. Palliative care should be interprofessional to cover the wide spectrum of needs that these patients will face.[20][21]

Enhancing Healthcare Team Outcomes

The mainstay of treatment for ependymoma includes an interprofessional approach that may include surgery, radiation therapy, and chemotherapy. For confirmation of the tumor through tissue biopsy or tumor resection therapy, referral to neurosurgery is necessary. Palliative care may be necessary for patients at end-of-life.[20][21] Those who undergo surgery may also develop a number of neurological deficits. These individuals may require physical therapy, speech therapy, and occupational therapy. In many cases, the neurological deficits are permanent.

Review Questions


Wu J, Armstrong TS, Gilbert MR. Biology and management of ependymomas. Neuro Oncol. 2016 Jul;18(7):902-13. [PMC free article: PMC4896548] [PubMed: 27022130]
Taylor MD, Poppleton H, Fuller C, Su X, Liu Y, Jensen P, Magdaleno S, Dalton J, Calabrese C, Board J, Macdonald T, Rutka J, Guha A, Gajjar A, Curran T, Gilbertson RJ. Radial glia cells are candidate stem cells of ependymoma. Cancer Cell. 2005 Oct;8(4):323-35. [PubMed: 16226707]
Mack SC, Taylor MD. The genetic and epigenetic basis of ependymoma. Childs Nerv Syst. 2009 Oct;25(10):1195-201. [PubMed: 19536551]
Leeper H, Felicella MM, Walbert T. Recent Advances in the Classification and Treatment of Ependymomas. Curr Treat Options Oncol. 2017 Aug 10;18(9):55. [PubMed: 28795287]
Ostrom QT, Gittleman H, Truitt G, Boscia A, Kruchko C, Barnholtz-Sloan JS. CBTRUS Statistical Report: Primary Brain and Other Central Nervous System Tumors Diagnosed in the United States in 2011-2015. Neuro Oncol. 2018 Oct 01;20(suppl_4):iv1-iv86. [PMC free article: PMC6129949] [PubMed: 30445539]
Brandão LA, Young Poussaint T. Posterior Fossa Tumors. Neuroimaging Clin N Am. 2017 Feb;27(1):1-37. [PubMed: 27889018]
Vitanza NA, Partap S. Pediatric Ependymoma. J Child Neurol. 2016 Oct;31(12):1354-66. [PubMed: 26503805]
Pajtler KW, Mack SC, Ramaswamy V, Smith CA, Witt H, Smith A, Hansford JR, von Hoff K, Wright KD, Hwang E, Frappaz D, Kanemura Y, Massimino M, Faure-Conter C, Modena P, Tabori U, Warren KE, Holland EC, Ichimura K, Giangaspero F, Castel D, von Deimling A, Kool M, Dirks PB, Grundy RG, Foreman NK, Gajjar A, Korshunov A, Finlay J, Gilbertson RJ, Ellison DW, Aldape KD, Merchant TE, Bouffet E, Pfister SM, Taylor MD. The current consensus on the clinical management of intracranial ependymoma and its distinct molecular variants. Acta Neuropathol. 2017 Jan;133(1):5-12. [PMC free article: PMC5209402] [PubMed: 27858204]
Maiuri F, Gangemi M, Iaconetta G, Signorelli F, Del Basso De Caro M. Symptomatic subependymomas of the lateral ventricles. Report of eight cases. Clin Neurol Neurosurg. 1997 Feb;99(1):17-22. [PubMed: 9107462]
Chhabda S, Carney O, D'Arco F, Jacques TS, Mankad K. The 2016 World Health Organization Classification of tumours of the Central Nervous System: what the paediatric neuroradiologist needs to know. Quant Imaging Med Surg. 2016 Oct;6(5):486-489. [PMC free article: PMC5130574] [PubMed: 27942466]
Louis DN, Perry A, Reifenberger G, von Deimling A, Figarella-Branger D, Cavenee WK, Ohgaki H, Wiestler OD, Kleihues P, Ellison DW. The 2016 World Health Organization Classification of Tumors of the Central Nervous System: a summary. Acta Neuropathol. 2016 Jun;131(6):803-20. [PubMed: 27157931]
Bouffet E, Perilongo G, Canete A, Massimino M. Intracranial ependymomas in children: a critical review of prognostic factors and a plea for cooperation. Med Pediatr Oncol. 1998 Jun;30(6):319-29; discussion 329-31. [PubMed: 9589080]
Cage TA, Clark AJ, Aranda D, Gupta N, Sun PP, Parsa AT, Auguste KI. A systematic review of treatment outcomes in pediatric patients with intracranial ependymomas. J Neurosurg Pediatr. 2013 Jun;11(6):673-81. [PubMed: 23540528]
Broniscer A, Ke W, Fuller CE, Wu J, Gajjar A, Kun LE. Second neoplasms in pediatric patients with primary central nervous system tumors: the St. Jude Children's Research Hospital experience. Cancer. 2004 May 15;100(10):2246-52. [PubMed: 15139071]
Spiegler BJ, Bouffet E, Greenberg ML, Rutka JT, Mabbott DJ. Change in neurocognitive functioning after treatment with cranial radiation in childhood. J Clin Oncol. 2004 Feb 15;22(4):706-13. [PubMed: 14966095]
Duffner PK, Krischer JP, Horowitz ME, Cohen ME, Burger PC, Friedman HS, Kun LE. Second malignancies in young children with primary brain tumors following treatment with prolonged postoperative chemotherapy and delayed irradiation: a Pediatric Oncology Group study. Ann Neurol. 1998 Sep;44(3):313-6. [PubMed: 9749596]
Stavrou T, Bromley CM, Nicholson HS, Byrne J, Packer RJ, Goldstein AM, Reaman GH. Prognostic factors and secondary malignancies in childhood medulloblastoma. J Pediatr Hematol Oncol. 2001 Oct;23(7):431-6. [PubMed: 11878577]
Cohen A, Rovelli A, Merlo DF, van Lint MT, Lanino E, Bresters D, Ceppi M, Bocchini V, Tichelli A, Socié G. Risk for secondary thyroid carcinoma after hematopoietic stem-cell transplantation: an EBMT Late Effects Working Party Study. J Clin Oncol. 2007 Jun 10;25(17):2449-54. [PubMed: 17557958]
Armstrong TS, Vera-Bolanos E, Gilbert MR. Clinical course of adult patients with ependymoma: results of the Adult Ependymoma Outcomes Project. Cancer. 2011 Nov 15;117(22):5133-41. [PubMed: 21538344]
Himelstein BP, Hilden JM, Boldt AM, Weissman D. Pediatric palliative care. N Engl J Med. 2004 Apr 22;350(17):1752-62. [PubMed: 15103002]
Gade G, Venohr I, Conner D, McGrady K, Beane J, Richardson RH, Williams MP, Liberson M, Blum M, Della Penna R. Impact of an inpatient palliative care team: a randomized control trial. J Palliat Med. 2008 Mar;11(2):180-90. [PubMed: 18333732]

Disclosure: Edgar Zamora declares no relevant financial relationships with ineligible companies.

Disclosure: Fahad Alkherayf declares no relevant financial relationships with ineligible companies.

Copyright © 2024, StatPearls Publishing LLC.

This book is distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International (CC BY-NC-ND 4.0) ( http://creativecommons.org/licenses/by-nc-nd/4.0/ ), which permits others to distribute the work, provided that the article is not altered or used commercially. You are not required to obtain permission to distribute this article, provided that you credit the author and journal.

Bookshelf ID: NBK538244PMID: 30855832


  • PubReader
  • Print View
  • Cite this Page

Related information

  • PMC
    PubMed Central citations
  • PubMed
    Links to PubMed

Similar articles in PubMed

See reviews...See all...

Recent Activity

Your browsing activity is empty.

Activity recording is turned off.

Turn recording back on

See more...