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Pediatric Ependymoma Follow-up

  • Author: Tobey J MacDonald, MD; Chief Editor: Max J Coppes, MD, PhD, MBA  more...
Updated: Nov 25, 2014

Further Outpatient Care

See the list below:

  • 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).
    • Liu et al conducted a retrospective review of a territory-wide database to determine the optimal timing of radiation therapy (RT) for children who were diagnosed with ependymoma between 1995 and 2011.[6] Overall survival and event-free survival were compared between patients receiving up-front RT (ie, RT performed in fewer than 150 days of diagnosis), delayed RT (performed 150 days or longer after diagnosis), or no RT. The study cohort consisted of 31 patients with intracranial ependymoma (mean age, 3.5 years; 45% male). The primary tumor was supratentorial in 10 patients (32%) and infratentorial in 21 (68%). All patients underwent initial surgery; gross total resection was performed in 27 patients (87%). Twelve patients (39%) received upfront RT, 10 (32%) received delayed RT, and 9 (29%) received no RT. During the study period, there were 11 relapses (35%) and 10 deaths (32%). Five-year overall survival was 69.9%; 5-year event-free survival was 49.3%. In univariate analysis, gross total resectionwas associated with improved overall survival event-free survival; OS and EFS was better patients who received RT than in those who did not. Upfront RT resulted in better OS and EFS than delayed RT. No significant effect on survival was observed with regard to age, sex, tumor location, RT dosage, and protocol used. The investigators recommended early initiation of adjuvant RT in the multimodal management of pediatric ependymomas.[6]
  • 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.

Further Inpatient Care

See the list below:

  • 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.

Inpatient & Outpatient Medications

See the list below:

  • 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.


See the list below:

  • 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.


See the list below:

  • 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


See the list below:

  • 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%).[7] 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.

Patient Education

See the list below:

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

Tobey J MacDonald, MD Professor, Department of Pediatrics, Emory University School of Medicine; Director, Pediatric Brain Tumor Program, Aflac Chair for Neuro-Oncology, Aflac Cancer and Blood Disorders Center, Children's Healthcare of Atlanta

Tobey J MacDonald, MD is a member of the following medical societies: American Association for Cancer Research, Society for Neuro-Oncology, International Society of Paediatric Oncology

Disclosure: Nothing to disclose.


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, Society for Neuro-Oncology, Pediatric Brain Tumor Consortium, Neurofibromatosis Clinical Trials Consortium

Disclosure: Nothing to disclose.

Specialty Editor Board

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, FAAP Chief, Division of Hematology/Oncology/BMT, Gordon Teter Endowed Chair in Pediatric Cancer, Nationwide Children's Hospital; Professor of Pediatrics, Ohio State University College of Medicine

Timothy P Cripe, MD, PhD, FAAP is a member of the following medical societies: American Academy of Pediatrics, American Association for the Advancement of Science, American Association for Cancer Research, American Pediatric Society, American Society of Gene and Cell Therapy, American Society of Pediatric Hematology/Oncology, Connective Tissue Oncology Society, Society for Pediatric Research, Children's Oncology Group

Disclosure: Nothing to disclose.

Chief Editor

Max J Coppes, MD, PhD, MBA Executive Vice President, Chief Medical and Academic Officer, Renown Heath

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

Disclosure: Nothing to disclose.


Samuel Gross, MD Professor Emeritus, Department of Pediatrics, University of Florida College of Medicine; Clinical Professor, Department of Pediatrics, University of North Carolina at Chapel Hill School of Medicine; Adjunct Professor, Department of Pediatrics, Duke University School of Medicine

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.

  1. Wang H, Zhang S, Rehman SK, Zhang Z, Li W, Makki MS, et al. Clinicopathological features of myxopapillary ependymoma. J Clin Neurosci. 2014 Apr. 21(4):569-73. [Medline].

  2. 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. 1993 Jun 17. 328(24):1725-31. [Medline].

  3. 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. 2011 Nov 12. [Medline].

  4. Ailon T, Dunham C, Carret AS, Tabori U, Mcneely PD, Zelcer S, et al. The role of resection alone in select children with intracranial ependymoma: the Canadian Pediatric Brain Tumour Consortium experience. Childs Nerv Syst. 2014 Nov 13. [Medline].

  5. 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. 2012 Jan 13. [Medline].

  6. Liu AP, Shing MM, Yuen HL, Li CH, Ling SC, Luk CW, et al. Timing of adjuvant radiotherapy and treatment outcome in childhood ependymoma. Pediatr Blood Cancer. 2014 Apr. 61(4):606-11. [Medline].

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

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

  9. 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. 2005 Oct 20. 23(30):7621-31. [Medline].

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

  11. 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. 2007 Aug. 8(8):696-705. [Medline].

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

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

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

  15. 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. 2004 Aug 1. 22(15):3156-62. [Medline].

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

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

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

  19. 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. 1998 Apr. 88(4):695-703. [Medline].

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

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

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

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

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

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

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

  27. Thorarinsdottir HK, Rood B, Kamani N, et al. Outcome for children 111111111111111111Pediatr Blood Cancer</i>. 2006 Feb 2. [Medline].

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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