Pediatric Ependymoma Workup

  • Author: Tobey MacDonald, MD; Chief Editor: Max J Coppes, MD, PhD, MBA   more...
 
Updated: Feb 29, 2012
 

Imaging Studies

The following studies are indicated in patients with ependymoma:

  • Head CT scan with and without contrast
    • This study has greater than 95% sensitivity for detection of brain tumors.
    • On CT scan, hydrocephalus is present in almost all patients.
    • The tumor typically appears as hyperdense and homogeneously contrast-enhancing lesion arising from, or adjacent to, the ventricular system.
    • Cystic areas and calcifications are frequent.
  • Head and spine MRI with and without gadolinium
    • These studies must be performed in all patients with CT scan or clinical findings consistent with ependymoma. Other tumors such as medulloblastoma and cerebellar astrocytoma may have similar appearance on CT scans. MRI can be useful in such instances by better demonstrating the anatomic origin and extent of tumor. With MRI, ependymoma is typically noted to be isointense to hypointense on T1-weighted images and hyperintense on T2-weighted images, relative to white matter. Ependymomas commonly show enhancement with gadolinium contrast. See the following image. MRI showing an ependymoma of the fourth ventricle,MRI showing an ependymoma of the fourth ventricle, compressing the cerebellum and brain stem.
    • MRI is the most sensitive means of detecting spinal cord lesions and should always be performed because of the potential for subarachnoid seeding, which is especially likely from tumors that arise in the posterior fossa.
    • A postoperative MRI is required both for the measurement of the extent of surgical resection and the detection of residual disease.
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Procedures

  • A CSF cytologic examination is useful for detection of microscopic leptomeningeal dissemination.
  • A CT scan or MRI must be performed prior to the lumbar puncture (LP) to exclude the presence of hydrocephaly. The presence of hydrocephaly would place the patient at risk for herniation as a consequence of the procedure. In general, the LP is deferred as long as 2 weeks postoperatively in order to avoid identifying tumor cells that may have been disseminated as a result of surgery.
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Histologic Findings

  • Ependymomas develop from the neuroepithelial lining of the ventricle and the central canal of the spinal cord. They are generally well-demarcated tumors that often display areas of calcification, hemorrhage, and cysts.
  • They vary morphologically from well differentiated with no anaplasia and little polymorphism (cellular or low-grade) to highly cellular lesions with significant mitotic activity, anaplasia, and necrosis, which may resemble glioblastoma multiforme (anaplastic or high-grade). See the image shown below. Section displaying high cellularity, nuclear atypiSection displaying high cellularity, nuclear atypia, and numerous mitoses characteristic of an anaplastic ependymoma.
  • Historically, they have been classified as cellular, epithelial, papillary, or mixed. However, this terminology is currently not used anymore. Tumors arising in the conus medullaris and filum terminale are termed myxopapillary because of their unique histopathologic features.
  • Ependymal rosettes are radially aligned, ependymal elements about a central lumen; although uncommon, they are a diagnostic feature of ependymoma. More common are pseudorosettes (see image below), an eosinophilic halo composed of cells with tapering processes surrounding a blood vessel. Section displaying typical perivascular pseudoroseSection displaying typical perivascular pseudorosettes of a benign ependymoma.
  • Electron microscopy may be useful in the diagnosis by demonstrating true rosettes, microvilli, and cilia on the apical surface. Immunohistochemistry and cytogenetic analysis have not been shown to demonstrate meaningful associations.
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Contributor Information and Disclosures
Author

Tobey MacDonald, MD  Clinical Director of Neuro-Oncology, Children's Hospital National Medical Center; Associate Professor, Department of Pediatric Hematology-Oncology, George Washington University

Tobey MacDonald, MD is a member of the following medical societies: American Association for Cancer Research, Children's Oncology Group, Pediatric Brain Tumor Consortium, and Society for Neuro-Oncology

Disclosure: Nothing to disclose.

Coauthor(s)

Roger J Packer, MD  Senior Vice President, Neuroscience and Behavioral Medicine, Director, Brain Tumor Institute, Children's National Medical CenterProfessor of Neurology and Pediatrics, The George Washington University

Roger J Packer, MD is a member of the following medical societies: American Academy of Neurology, American Neurological Association, American Pediatric Society, Child Neurology Society, Children's Oncology Group, Neurofibromatosis Clinical Trials Consortium, Pediatric Brain Tumor Consortium, and Society for Neuro-Oncology

Disclosure: Nothing to disclose.

Specialty Editor Board

Samuel Gross, MD  Professor Emeritus, Department of Pediatrics, University of Florida; Clinical Professor, Department of Pediatrics, University of North Carolina; Adjunct Professor, Department of Pediatrics, Duke University

Samuel Gross, MD is a member of the following medical societies: American Association for Cancer Research, American Society for Blood and Marrow Transplantation, American Society of Clinical Oncology, American Society of Hematology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Timothy P Cripe, MD, PhD  Professor of Pediatrics, Division of Hematology/Oncology, Cincinnati Children's Hospital Medical Center; Clinical Director, Musculoskeletal Tumor Program, Co-Medical Director, Office for Clinical and Translational Research, Cincinnati Children's Hospital Medical Center; Director of Pilot and Collaborative Clinical and Translational Studies Core, Center for Clinical and Translational Science and Training, University of Cincinnati College of Medicine

Timothy P Cripe, MD, PhD is a member of the following medical societies: American Association for the Advancement of Science, American Pediatric Society, American Society of Hematology, American Society of Pediatric Hematology/Oncology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

David Pallares, MD  Clinical Assistant Professor, Department of Pediatrics, Division of Allergy and Immunology, University of Louisville School of Medicine

David Pallares, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology

Disclosure: Nothing to disclose.

Chief Editor

Max J Coppes, MD, PhD, MBA  Senior Vice President, Center for Cancer and Blood Disorders, Children's National Medical Center; Professor of Medicine, Oncology, and Pediatrics, Georgetown University School of Medicine; Clinical Professor of Pediatrics, George Washington University School of Medicine and Health Sciences

Max J Coppes, MD, PhD, MBA is a member of the following medical societies: American Association for Cancer Research, American Society of Pediatric Hematology/Oncology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

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MRI showing an ependymoma of the fourth ventricle, compressing the cerebellum and brain stem.
Sagittal section of an ependymoma of the fourth ventricle.
Section displaying typical perivascular pseudorosettes of a benign ependymoma.
Section displaying high cellularity, nuclear atypia, and numerous mitoses characteristic of an anaplastic ependymoma.
 
 
 
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