Pediatric Ependymoma Treatment & Management

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

Medical Care

  • Surgery: Surgery is the initial treatment for ependymoma. Patients with total or near total resections have significantly better survival rates than those in whom the resection is grossly incomplete.
  • Radiotherapy: This remains the standard postoperative therapy. Whether this improves long-term survival remains controversial, especially in infants and those with completely resected tumors, in whom the risk of long-term effects of radiotherapy may outweigh the potential benefits.
  • The role of chemotherapy is unknown. Clinical trials evaluating the effectiveness of different chemotherapeutic agents for infants and for subtotally resected tumors are ongoing. Promising results have been demonstrated with cyclical oral etoposide in recurrent ependymoma, with response rates as high as 83%. However, induction of secondary leukemia in association with chronic use of this regimen has been reported.
  • Involved-field radiotherapy
    • In patients with complete resection, 59.4 Gy (infratentorial tumor) and 55.8 Gy (supratentorial tumor) to the original tumor site plus a 1-2 cm margin is recommended.
    • In patients with subtotal resection, 59.4 Gy to the original tumor site plus a 1-2 cm margin for supratentorial and infratentorial tumors is recommended.
    • Craniospinal radiation is recommended only for those with leptomeningeal dissemination.
    • Conformal radiotherapy has been shown to be effective for those with localized, totally resected posterior fossa tumors.
  • Infants younger than 3 years: Studies attempting to delay or omit radiotherapy by using postoperative chemotherapy are ongoing.[1, 2]
  • Chemotherapy
    • Although with current regimens the role of chemotherapy appears limited, measurable responses have been documented.
    • As a single agent, cisplatin has been the most effective in phase II studies (30% response rate).
    • Other platinum compounds such as carboplatin appear less effective.
    • One study has reported significant responses to vincristine and cyclophosphamide combined.
    • Trials investigating the effectiveness of preirradiation chemotherapy using platinum or alkylator-based regimens are ongoing in infants and patients with subtotally resected tumors. Preliminary results suggest some benefit in those with anaplastic tumors.
    • A Children's Oncology Group study evaluated the benefits of preirradiation multiagent chemotherapy in patients with subtotally resected tumors.
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Surgical Care

  • The goal is for gross total or near total resection.
  • Posterior fossa tumors are approached through a suboccipital craniotomy and may be incompletely resectable because of infiltration of the floor, the fourth ventricle, or brainstem.
  • Supratentorial tumors tend to be large and may be intraventricular, extraventricular, or both. These tumors have a predilection for the frontal, temporal, and parietal lobes and third ventricle. Approach and degree of resection depend on the tumor's size and location.
  • A regional, leptomeningeal examination must be examined for metastatic foci.
  • Surgical estimates of the extent of resection may not be reliable; therefore, postoperative MRI evaluation for residual disease is required and generally should be performed within 72 hours of surgery to avoid confusion with postsurgical inflammation.
  • Posterior fossa tumors often present with obstructive hydrocephaly and may require the placement of a ventriculoperitoneal shunt if primary resection to reestablish CSF flow is unsuccessful.
  • The use of "second-look" surgery after 1-2 cycles of chemotherapy before radiotherapy has been studied, and the results are pending.
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Consultations

  • Regular team members for the care of all patients include the following:
    • Neurosurgeon
    • Radiation oncologist
    • Pediatric oncologist or neuro-oncologist
    • Neurologist
    • Neuropsychologist
    • Neuroendocrinologist
  • As a result of the tumor, therapeutic intervention, or both, some patients may require the assistance of occupational and physical therapists for rehabilitation.
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Diet

  • No specific dietary restrictions or requirements are indicated.
  • Patients who develop severe anorexia or weight loss as a result of therapy (particularly infants) may need supplemental nutrition to maintain daily requirements.
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Activity

  • No activity restrictions are required unless dictated by underlying neurological deficits.
  • Patients with ventriculoperitoneal shunts may be restricted from high-impact sports such as diving.
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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.

References

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  2. Sung KW, Lim DH, Lee SH, Yoo KH, Koo HH, Kim JH, et al. Tandem high-dose chemotherapy and autologous stem cell transplantation for anaplastic ependymoma in children younger than 3 years of age. J Neurooncol. Nov 12 2011;[Medline].

  3. Bouffet E, Hawkins CE, Balloura W, Taylor MD, Bartels UK, Schoenhoff N, et al. Survival Benefit for Pediatric Patients with Recurrent Ependymoma Treated with Reirradiation. Int J Radiat Oncol Biol Phys. Jan 13 2012;[Medline].

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  7. Goldwein JW, Glauser TA, Packer RJ. Recurrent intracranial ependymomas in children. Survival, patterns of failure, and prognostic factors. - Packer RJ. Aug 1 1990;66(3):557-63. [Medline].

  8. Grundy RG, Wilne SA, Weston CL, et al. Primary postoperative chemotherapy without radiotherapy for intracranial ependymoma in children: the UKCCSG/SIOP prospective study. Lancet Oncol. Aug 2007;8(8):696-705. [Medline].

  9. Heideman RL, Packer RJ, Albright LA. Tumors of the central nervous system. In: Principles and Practice of Pediatric Oncology. 3rd ed. Raven Press; 1997:633-97.

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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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