Guidelines Summary

The National Comprehensive Cancer Network (NCCN) recommends the following for treatment of adults with ependymoma^[3]:

Following suspicion for ependymoma on neuroimaging, maximal safe resection should be performed
Following diagnosis of grade 2 or grade 3 ependymoma after resection/biopsy, conduct brain and spine MRI, plus LP to assess for leptomeningeal spread (LP contraindicated with posterior fossa masses)
If post-resection neuroimaging is negative for metastasis, administer standard conformal radiation therapy (to tumor area plus 1-2cm margins). If metastasis detected, administer whole craniospinal radiation therapy (or proton therapy to reduce toxicity.) Spinal ependymomas < WHO Grade 2 do not require radiation therapy if gross total resection was performed and neuroimaging/LP are negative.
MRI surveillance for recurrence following resection for 5-10 years
For recurrent ependymoma, patients who have not received radiation therapy should receive radiation therapy, and if a patient has received radiation therapy, then chemotherapy, radiation therapy, or supportive care should be considered.

The European Association of Neuro-oncology (EANO) recommends a similar treatment approach to the NCCN, advocating for maximal safe resection followed by post operative radiation and monitoring for recurrence. However, the EANO recommends adjuvant chemotherapy (exact regimen varies) instead of radiation in patients less than 12 months old to avoid harmful side effects of radiotherapy. On the contrary, in the NCCN guidelines, chemotherapy is not recommended as a monotherapy and is only considered in recurrent cases where resection and radiation have failed.^[68]

Medication

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Media Gallery

CT scan without contrast. Fourth ventricle ependymoma.
CT scan without contrast. Fourth ventricle ependymoma. Note blood in the fourth ventricle.
CT scan without contrast in the patient with fourth ventricle ependymoma. Blood has refluxed into the third and lateral ventricles.
CT scan without contrast in the patient with fourth ventricle ependymoma. Note blood traversing foramina.
T1-weighted MRI. Rare case of a fourth ventricle ependymoma presenting as an intraventricular bleed.
T1-weighted MRI without contrast demonstrating ependymoma located in the fourth ventricle.
T2-weighted MRI demonstrating ependymoma in the fourth ventricle.
Coronal T1-weighted MRI with contrast demonstrating ependymoma of the fourth ventricle.
Gross surgical specimen of a fourth ventricle ependymoma.
Histologic study of a classic ependymoma. Note the characteristic perivascular pseudorosettes.
Cellular ependymoma. Cells with a high nuclear-cytoplasmic ratio. Few pseudorosettes or paucicellular areas are present.
Myxopapillary ependymoma. Clusters of loosely arranged cuboidal cells separated by pools of mucin.
Clear cell ependymoma. Round cells with cytoplasmic clearing. This may mimic an oligodendroglioma.
MRI images of an ependymoma in the left ventricle. Courtesy of figshare.com [El Majdoub, Faycal; Elawady, Moataz; Blau, Tobias; Bührle, Christian; Hoevels, Mauritius; Runge, Matthias; et al. (2016): Intracranial Ependymoma: Long-Term Results in a Series of 21 Patients Treated with Stereotactic 125Iodine Brachytherapy. PLOS ONE. Dataset. Available at: https://figshare.com/articles/dataset/Intracranial_Ependymoma_Long_Term_Results_in_a_Series_of_21_Patients_Treated_with_Stereotactic_125_Iodine_Brachytherapy__/117659].
MRI image of the sagittal neck with an ependymoma. Modification of a figure from nl-wiki, without author annotations. Courtesy of Wikimedia Commons [Author Lucien Monfils, available at: https://commons.wikimedia.org/wiki/File:Ependymoma.png].

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Author

Jeffrey N Bruce, MD Edgar M Housepian Professor of Neurological Surgery Research, Vice-Chairman and Professor of Neurological Surgery, Director of Columbia Brain Tumor Center, Director of Bartoli Brain Tumor Laboratory, Department of Neurosurgery, Columbia University Vagelos College of Physicians and Surgeons

Jeffrey N Bruce, MD is a member of the following medical societies: Alpha Omega Alpha, American Association of Neurological Surgeons, American Society of Clinical Oncology, Congress of Neurological Surgeons, New York Academy of Sciences, North American Skull Base Society, Pituitary Society, Society for Neuro-Oncology, Society of Neurological Surgeons

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Theracle, Inc. <br/>Received grant/research funds from NIH for other.

Coauthor(s)

Adithya Kannan, BS MD Candidate, Columbia University Vagelos College of Physicians and Surgeons

Disclosure: Nothing to disclose.

Nadine M Khoury MD Candidate, Columbia University Vagelos College of Physicians and Surgeons

Disclosure: Received research grant from: NIH.

Nina Teresa Yoh, MD Resident Physician, Department of Neurological Surgery, Columbia University Irving Medical Center, New York-Presbyterian Hospital

Nina Teresa Yoh, MD is a member of the following medical societies: Alpha Omega Alpha, American Association of Neurological Surgeons, Congress of Neurological Surgeons, Gold Humanism Honor Society, Neurocritical Care Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

Herbert H Engelhard, III, MD, PhD, FACS, FAANS Affiliated Professor of Bioengineering, University of Illinois at Chicago

Herbert H Engelhard, III, MD, PhD, FACS, FAANS is a member of the following medical societies: American Association for Cancer Research, American Association of Neurological Surgeons, American College of Surgeons, American Medical Association, Congress of Neurological Surgeons, Illinois State Medical Society

Disclosure: Nothing to disclose.

Additional Contributors

Robert C Shepard, MD, FACP Associate Professor of Medicine in Hematology and Oncology at University of North Carolina at Chapel Hill; Vice President of Scientific Affairs, Therapeutic Expertise, Oncology, at PRA International

Robert C Shepard, MD, FACP is a member of the following medical societies: American Association for Cancer Research, American Association for Physician Leadership, European Society for Medical Oncology, Association of Clinical Research Professionals, American Federation for Clinical Research, Eastern Cooperative Oncology Group, Society for Immunotherapy of Cancer, American Medical Informatics Association, American College of Physicians, American Federation for Medical Research, American Medical Association, American Society of Hematology, Massachusetts Medical Society

Disclosure: Nothing to disclose.

Neil A Feldstein, MD Director of Pediatric Neurosurgery, Department of Neurosurgery, Babies and Children's Hospital of New York, Assistant Professor,Departments of Clinical Neurosurgery and Pediatrics, Columbia-Presbyterian Medical Center, Columbia University

Neil A Feldstein, MD is a member of the following medical societies: American Association of Neurological Surgeons, American Cleft Palate-Craniofacial Association, American College of Surgeons, American Medical Association, Medical Society of the State of New York

Disclosure: Nothing to disclose.

David J Fusco, MD Neurosurgeon and Partner, Barrow Brain and Spine; Director of Inpatient Neurosurgical Care, Chandler Regional Medical Center

David J Fusco, MD is a member of the following medical societies: American Association of Neurological Surgeons, American Medical Association, Congress of Neurological Surgeons, North American Spine Society

Disclosure: Nothing to disclose.

Benjamin C Kennedy, MD Assistant Professor of Neurosurgery, Perelman School of Medicine at the University of Pennsylvania; Director of Epilepsy and Functional Neurosurgery, The Children’s Hospital of Philadelphia

Disclosure: Nothing to disclose.

Acknowledgements

We wish to acknowledge previous contributions to this article by Paul C McCormick, MD, and Allen Waziri, MD.

Supratentorial ependymomas	ZFTA (C11orf95): Zinc Finger Translocation Associated gene
Supratentorial ependymomas	YAP1: Yes-Associated Protein 1
Posterior fossa ependymomas	Posterior Fossa Group A molecular profile
Posterior fossa ependymomas	Posterior Fossa Group B molecular profile
Spinal ependymomas	NF2: Neurofibromatosis 2 gene
	MYCN Proto-oncogene
	MYCN Non-amplified
Subependymoma	Supratentorial
	Posterior fossa
	Spinal

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