Pediatric Ependymoma Clinical Presentation

Updated: Aug 22, 2017
  • Author: Eugene I Hwang, MD; Chief Editor: Max J Coppes, MD, PhD, MBA  more...
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Presentation

History

The initial presentation for ependymoma in childhood varies according to the location(s) of the ependymoma. Most children present with non-specific symptoms related to increased intracranial pressure.   

  • The classic triad of raised ICP consists of morning headaches, vomiting, and lethargy. The classic headache of increased ICP is pain present upon rising, relieved by vomiting, and gradually decreasing during the day.
  • School-aged children more commonly complain of vague, intermittent headaches and fatigue. They may demonstrate declining academic performance and exhibit personality changes.
  • Infants may present with irritability, anorexia, and developmental delay or regression.
  • Supratentorial lesions may be associated with seizures and focal cerebral deficits.
  • Posterior fossa tumors may lead to cerebellar dysfunction, resulting in balance and gait disturbances that frequently are associated with vomiting and lower cranial nerve findings such as diplopia, facial weakness, tinnitus, vertigo, and hearing loss.
  • Spinal cord tumors may cause symptoms of spinal cord compression, such as back pain and loss of bladder and/or bowel control.
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Physical

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  • Increased ICP findings
    • A funduscopic examination reveals papilledema. Infants may have only optic pallor.
    • Palsy of cranial nerve VI, resulting in the inability to abduct one or both eyes, is common.
    • Infants may demonstrate the setting sun sign, observed as an impaired upgaze and a forced downward deviation of both eyes. Measurement of head circumference in infants with open cranial sutures may reveal macrocephaly.
  • Localized deficits in truncal steadiness, upper extremity coordination, and gait may be observed with posterior fossa tumors.
  • The inability to deviate both eyes conjugatively (gaze palsy), or the inability to adduct one eye on attempted lateral gaze may be seen with tumor invasion into the brainstem.
  • Extension of posterior fossa tumors through the foramina of Luschka may impair function of the lower cranial nerves (primarily VI, VII, VIII, IX, and X).
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Causes

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  • Epidemiological studies investigating parental occupational exposures, environmental exposures, and maternal nutritional intake have failed to identify linkages with any of the childhood brain tumors.
  • DNA sequences similar to SV40 virus and the virus-encoded large T-antigen have been found in several ependymomas, but no conclusions regarding causation have been determined. SV40 and related polyoma viruses can induce ependymoma in monkeys and other mammalian species.
  • Neurofibromatosis type 2 (NF2) is a genetic disorder that is known to predispose to ependymoma, typically spinal tumors.
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