Ependymoma represents the third most common primary brain tumor in children and accounts for approximately 10% of brain tumors in children and >20% of spinal cord tumors in children .
Ependymoma diagnosis peaks between 0 and 4 years of age, and ependymomas are slightly more common in boys than in girls. Overall, they affect 0.26/100,000 children aged 0–14 years [1, 2].
- Ependymoma may be further characterized according to histopathology as well as location within the CNS (supratentorial brain, infratentorial brain, spinal cord).
The World Health Organization (WHO) separates ependymomas into three groups based on histopathologic grade, with several subclassifications. Subclassifications have been recently modified with a 2016 update of the WHO CNS 4th edition; modifications include incorporation of a genetically defined ependymoma variant (RELA fusion-positive). (Table 4.1) .
RELA fusion-positive ependymoma is the first molecular ependymoma variant to be formally recognized. Over two-thirds of supratentorial ependymomas contain oncogenic fusions between RELA (an effector of NF-κB signaling) and an uncharacterized gene, C11orf95, involving chromosome 11q13 .
Additionally, children are affected by these tumors within specific anatomic areas based on age, with mean age 12.2 years for spinal cord ependymomas, 7.8 years for supratentorial tumors, and 5 years for infratentorial tumors .
Ependymomas can disseminate throughout the central nervous system, although this is relatively rare, with <15% of ependymoma tumors being associated with dissemination detected either on imaging or via lumbar puncture [6, 7]
- Surgical resection and focal radiotherapy remain the mainstays of treatment for ependymoma, with the role of chemotherapy remaining investigational.
Historically, surgical resection has been regarded as the most crucial aspect of treatment, with improvement in prognosis observed for patients with gross total as compared to incomplete resections [8–10].
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