Pediatric Ependymoma Clinical Presentation

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

History

  • Initial symptoms of ependymoma are usually nonspecific, nonlocalizing, and related to increased intracranial pressure (ICP).
    • The classic triad of raised ICP consists of morning headaches, vomiting, and lethargy. The classic headache of increased ICP is pain present upon rising, relieved by vomiting, and gradually decreasing during the day.
    • School-aged children more commonly complain of vague, intermittent headaches and fatigue. They may demonstrate declining academic performance and exhibit personality changes.
    • Infants may present with irritability, anorexia, and developmental delay or regression.
  • Supratentorial lesions may be associated with seizures and focal cerebral deficits.
  • Posterior fossa tumors may lead to cerebellar dysfunction, resulting in balance and gait disturbances that frequently are associated with vomiting and lower cranial nerve findings such as diplopia, facial weakness, tinnitus, vertigo, and hearing loss.
  • Spinal cord tumors may cause symptoms of spinal cord compression, such as back pain and loss of bladder and/or bowel control.
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Physical

  • Increased ICP findings
    • A funduscopic examination reveals papilledema. Infants may have only optic pallor.
    • Palsy of cranial nerve VI, resulting in the inability to abduct one or both eyes, is common.
    • Infants may demonstrate the setting sun sign, observed as an impaired upgaze and a forced downward deviation of both eyes. Measurement of head circumference in infants with open cranial sutures may reveal macrocephaly.
  • Localized deficits in truncal steadiness, upper extremity coordination, and gait may be observed with posterior fossa tumors.
  • The inability to deviate both eyes conjugatively (gaze palsy), or the inability to adduct one eye on attempted lateral gaze may be seen with tumor invasion into the brainstem.
  • Extension of posterior fossa tumors through the foramina of Luschka may impair function of the lower cranial nerves (primarily VI, VII, VIII, IX, and X).
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Causes

  • Epidemiological studies investigating parental occupational exposures, environmental exposures, and maternal nutritional intake have failed to identify linkages with any of the childhood brain tumors.
  • DNA sequences similar to SV40 virus and the virus-encoded large T-antigen have been found in several ependymomas, but no conclusions regarding causation have been determined. SV40 and related polyoma viruses can induce ependymoma in monkeys and other mammalian species.
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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

  1. Duffner PK, Horowitz ME, Krischer JP. Postoperative chemotherapy and delayed radiation in children less than three years of age with malignant brain tumors [see comments]. N Engl J Med. Jun 17 1993;328(24):1725-31. [Medline].

  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].

  4. Grill J, Pascal C, Chantal K. Childhood ependymoma: a systematic review of treatment options and strategies. Paediatr Drugs. 2003;5(8):533-43. [Medline].

  5. Bouffet E, Perilongo G, Canete A. Intracranial ependymomas in children: a critical review of prognostic factors and a plea for cooperation. Med Pediatr Oncol. Jun 1998;30(6):319-29; discussion 329-31. [Medline].

  6. Geyer JR, Sposto R, Jennings M, et al. Multiagent chemotherapy and deferred radiotherapy in infants with malignant brain tumors: a report from the Children's Cancer Group. J Clin Oncol. Oct 20 2005;23(30):7621-31. [Medline].

  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.

  10. Merchant TE, Boop FA, Kun LE, Sanford RA. A retrospective study of surgery and reirradiation for recurrent ependymoma. Int J Radiat Oncol Biol Phys. May 1 2008;71(1):87-97. [Medline].

  11. Merchant TE, Fouladi M. Ependymoma: new therapeutic approaches including radiation and chemotherapy. J Neurooncol. Dec 2005;75(3):287-99. [Medline].

  12. Merchant TE, Mulhern RK, Krasin MJ, et al. Preliminary results from a phase II trial of conformal radiation therapy and evaluation of radiation-related CNS effects for pediatric patients with localized ependymoma. J Clin Oncol. Aug 1 2004;22(15):3156-62. [Medline].

  13. Nazar GB, Hoffman HJ, Becker LE. Infratentorial ependymomas in childhood: prognostic factors and treatment. J Neurosurg. Mar 1990;72(3):408-17. [Medline].

  14. Pollack IF, Gerszten PC, Martinez AJ. Intracranial ependymomas of childhood: long-term outcome and prognostic factors. Neurosurgery. Oct 1995;37(4):655-66; discussion 666-7. [Medline].

  15. Reddy AT, Packer RJ. Pediatric central nervous system tumors. Curr Opin Oncol. May 1998;10(3):186-93. [Medline].

  16. Robertson PL, Zeltzer PM, Boyett JM, et al. Survival and prognostic factors following radiation therapy and chemotherapy for ependymomas in children: a report of the Children's Cancer Group. J Neurosurg. Apr 1998;88(4):695-703. [Medline].

  17. Sandri A, Massimino M, Mastrodicasa L, et al. Treatment with oral etoposide for childhood recurrent ependymomas. J Pediatr Hematol Oncol. Sep 2005;27(9):486-90. [Medline].

  18. Sexauer CL, Khan A, Burger PC. Cisplatin in recurrent pediatric brain tumors. A POG Phase II study. A Pediatric Oncology Group Study. Cancer. Oct 1 1985;56(7):1497-501. [Medline].

  19. Shu HK, Sall WF, Maity A, et al. Childhood intracranial ependymoma: twenty-year experience from a single institution. Cancer. Jul 15 2007;110(2):432-41. [Medline].

  20. Shuman RM, Alvord EC Jr, Leech RW. The biology of childhood ependymomas. Arch Neurol. Nov 1975;32(11):731-9. [Medline].

  21. Stratton MR, Darling J, Lantos PL. Cytogenetic abnormalities in human ependymomas. Int J Cancer. Oct 15 1989;44(4):579-81. [Medline].

  22. Tabori U, Ma J, Carter M, et al. Human telomere reverse transcriptase expression predicts progression and survival in pediatric intracranial ependymoma. J Clin Oncol. Apr 1 2006;24(10):1522-8. [Medline].

  23. Taylor MD, Poppleton H, Fuller C, et al. Radial glia cells are candidate stem cells of ependymoma. Cancer Cell. Oct 2005;8(4):323-35. [Medline].

  24. Thorarinsdottir HK, Rood B, Kamani N, et al. Outcome for children < 4 years of age with malignant central nervous system tumors treated with high-dose chemotherapy and autologous stem cell rescue. Pediatr Blood Cancer. Feb 2 2006;[Medline].

 
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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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