Ependymoma (patient information)

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Ependymoma
Overview
What are the symptoms?
What are the causes?
Who is at highest risk?
Diagnosis
When to seek urgent medical care?
Treatment options
Where to find medical care for Ependymoma?
Prevention
What to expect (Outlook/Prognosis)?
Possible complications
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Directions to Hospitals Treating Ependymoma
Risk calculators and risk factors for Ependymoma

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Ependymoma is the third most common neuroepithelial tumor of the central nervous system (CNS) in childhood. It arises for ependymal cells of the central nervous system and is dominated by intracranial mass. The World Health Organization (WHO) classification of CNS tumors defines several histopathological variants of ependymoma (grade I, II, III). On gross pathology, a well-encapsulated tumor arises from the floor of the fourth ventricle, situated in the lower back portion of the brain is a characteristic finding of ependymoma. On microscopic histopathological analysis, perivascular pseudorosettes are characteristic findings of ependymoma. Development of ependymoma is the result of multiple genetic mutations (ERBB2, ERBB4, MMP2, MMP14, NOTCH1, and MEN1). There are no established causes for ependymomas. Ependymoma must be differentiated from medulloblastoma, choroid plexus papilloma, and glioblastoma. Common risk factors in the development of ependymoma are children with certain hereditary diseases (neurofibromatosis type II and Turcot syndrome), ERBB2, ERBB4, and human telomerase reverse transcriptase TERT gene expression, over-expression of kinetochore proteins, and down-regulation of metallothioneins.Symptoms of ependymoma include headache, nausea, vomiting, blurry or double vision, drowsiness (after several hours of the above symptoms), irritability, ataxia, neck pain, cranial nerve palsies, seizures, focal neurologic deficits, back pain, lower extremity weakness, bowel and bladder dysfunction. MRI may be diagnostic of ependymoma. Finding on brain MRI suggestive of ependymoma include large mixed cystic/solid lesion with haemorrhage and fluid which may indicate areas of necrosis. The predominant therapy for ependymoma is surgical resection. Adjunctive chemoradiation may be required.

What are the symptoms of Ependymoma?

Symptoms of ependymoma include:

• Headache
• Nausea
• Vomiting
• Blurry or double vision
• Drowsiness (after several hours of the above symptoms)
• Irritability
• Ataxia
• Neck pain
• Cranial nerve palsies
• Seizures
• Focal neurologic deficits.
• Back pain
• Lower extremity weakness
• Bowel and bladder dysfunction

What causes Ependymoma?

• There are no established causes for ependymoma.

Who is at highest risk?

Children with certain hereditary diseases, such as neurofibromatosis type II (NF2), Turcot syndrome B, and MEN1 syndrome, have been found to be more frequently afflicted with ependymal tumors.

• Increased occurrence of chromosome 1q and proteins such as tenascin C and epidermal growth factor are associated with increased risk for developing ependymal tumors.
• ERBB2, ERBB4, and human telomerase reverse transcriptase TERT gene expression promote tumor cell proliferation, contributing to aggressive tumor behavior.
• High expression of epidermal growth factor receptor EGFR correlates with unfavorable outcome.
• Over-expression of kinetochore proteins and down-regulation of metallothioneins are associated with recurrence of ependymomas.

Treatment options

The predominant therapy for ependymoma is surgical resection. Adjunctive chemoradiation may be required.

Chemotherapy

• There is no evidence to date that adjuvant chemotherapy, including the use of myeloablative chemotherapy, improves the outcome for patients with totally resected, nondisseminated ependymoma.
• Current treatment approaches do not include chemotherapy as a standard component of primary therapy for children with newly diagnosed ependymomas that are completely resected.
• Etoposide may be used in recurrent cases.

Radiation Therapy

• Radiation therapy consisting of 54 gray to 55.8 gray is recommended for children aged 3 years and older who are diagnosed with well-differentiated ependymomas.
• It is not necessary to treat the entire CNS (whole brain and spine) because these tumors usually recur initially at the local site.
• When possible, patients should be treated in a center experienced with the delivery of highly conformal radiation therapy (including intensity-modulated radiation therapy or charged-particle radiation therapy) to pediatric patients with brain tumors.

Where to find medical care for Ependymoma?

Directions to Hospitals Treating Ependymoma