Ependymoma (patient information)
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Ependymoma |
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Ependymoma On the Web |
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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?
Who is at highest risk?
Diagnosis
When to seek urgent medical care?
Treatment options
Where to find medical care for Ependymoma?
Directions to Hospitals Treating Ependymoma