Pediatric Ependymoma Follow-up

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

Further Inpatient Care

  • Admit only patients with ependymoma who are eligible for investigational chemotherapy.
  • Investigational chemotherapy may cause complications such as fever, neutropenia, or suspected infection; therefore, hospitalization may be necessary.
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Further Outpatient Care

  • Radiotherapy: After the patient recovers from surgery, daily outpatient radiotherapy should begin. This is generally given for approximately 6 weeks (usual dose is 160-180 cGy per day).
  • Physical and neurologic examination
    • Monitoring of clinical response and potential treatment-related side effects should be on a weekly basis during radiotherapy. Protocols using investigational chemotherapy regimens dictate how frequently these examinations are conducted during treatment.
    • Following completion of therapy, assessments are generally performed every 3 months for the first year to 18 months, then every 6 months for the next 2 years, and annually thereafter, provided no interim complications occur.
    • Baseline neuropsychology and developmental testing should be performed at the completion of therapy and annually thereafter.
  • Imaging studies
    • An MRI with contrast of the head should be obtained at the completion of radiotherapy and then generally in conjuncture with the physical and neurologic examination schedule or sooner if clinically indicated.
      • Although the optimal timing of posttreatment imaging for the evaluation of both response to therapy and recurrence has yet to be determined, most clinicians agree that routine surveillance should be performed at least every 3-6 months during the first 2 years and every 6-12 months for the following 2-3 years after treatment.
      • Further MRI evaluations at 3-year to 5-year intervals may be useful for the detection of late events such as radiation-induced secondary tumors. Investigational chemotherapeutic regimens also may dictate the imaging study schedules.
    • An MRI of the spine should be obtained at the completion of treatment and then once yearly for the first 2 years after therapy, unless there is evidence of leptomeningeal dissemination at diagnosis or during therapy, in which case the frequency of such tests is increased in accordance with the response to treatment. Routine spinal evaluations beyond 2 years from the completion of treatment may not be practical since local recurrences are far more likely than isolated neuraxial disease.
  • Laboratory studies: A weekly CBC count during radiotherapy (to monitor for hematopoietic toxicity and to determine whether intervention should be carried out to maintain hemoglobin levels at or higher than 9 g/dL to maximize radiation effect) is all that is required unless dictated by investigational chemotherapeutic regimens or clinically indicated.
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Inpatient & Outpatient Medications

  • No medications are needed unless the patient is enrolled in an investigational chemotherapeutic regimen.
  • Dexamethasone may be necessary to reduce the inflammatory response associated with the tumor and/or therapy.
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Transfer

  • Transfer the patient to a pediatric center that can provide appropriate MRI imaging studies, neurosurgical intervention, and radiotherapy. Follow-up with a neuro-oncologist may be necessary.
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Complications

  • Obstructive hydrocephaly
  • Neurologic impairment
  • Radiation-induced effects
    • Neurocognitive decline
    • Endocrinologic dysfunction
    • Mineralizing microangiopathy with ischemia or infarct
    • Secondary CNS malignancies
    • Transient headaches, fatigue, nausea, vomiting, and anorexia
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Prognosis

  • Extent of tumor resection: Resection is the most important prognostic factor. Patients with gross total and near-total resections have reported survival rates of 51-80%, versus 0-26% in those with subtotal resections (< 90% removal of total tumor mass, visible tumor present on MRI).
  • Age: Very young patients (< 1 y), unrelated to radiation treatment, have a significantly worse prognosis (5-y survival rate of 25%).[4] The 5-year survival rate for children aged 1-4 years is also significantly less than for children older than 5 years (46% versus >70%). Some promising results using high-dose chemotherapy and delayed or omitted radiotherapy have been recently shown in this age group.
  • Other factors: Historically, anaplastic features and supratentorial location have conferred a worse prognosis. More recent reports have largely dismissed histology and tumor location as significant prognostic indicators (with the exception of better outcome observed in spinal cord tumors and myxopapillary tumors of the cauda equina). Metastatic disease is probably a poor prognostic factor; however, patient numbers are too scarce to draw a conclusion.
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Patient Education

  • The patient and his/her family members should be referred for psychosocial counseling.
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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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