Abstract
Ependymoma can arise throughout the whole neuraxis. In children, tumors predominantly
occur intracranially, whereas the spine is the most prevalent location in adults.
Significant variance in the grade II versus grade III distinction of ependymomas has
led to the acknowledgment that the clinical utility of histopathological classification
is limited. Epigenomic profiling efforts have identified molecularly distinct groups
of ependymomas that adequately reflect the biological, clinical, and histopathological
heterogeneities across anatomical compartments, age groups, and grades. The recent
update of the World Health Organization classification of central nervous system tumors
has already integrated one of these groups, and molecular classification will be part
of future clinical trials to improve risk stratification. Clinical management of this
rare disease is challenging, making professional experience and intensified multidisciplinary
cooperation pivotal factors for treatment success. Novel research strategies are currently
applied for target discovery in ependymomas since for most molecular groups, genetic
drivers are unknown.
Keywords
ependymoma - molecular classification - treatment - adult - children
