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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

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  • 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.
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Further Inpatient Care

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  • 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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Inpatient & Outpatient Medications

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  • 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

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  • 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

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  • 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

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  • 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.
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Patient Education

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

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.

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, 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.

Acknowledgements

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.

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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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