Abstract
Brain tumors account for the majority of cancer-related deaths in adolescents and
young adults (AYAs), defined as individuals aged 15 to 39. AYAs constitute a distinct
population in which both pediatric- and adult-type central nervous system (CNS) tumors
can be observed. Clinical manifestations vary depending on tumor location and often
include headaches, seizures, focal neurological deficits, and signs of increased intracranial
pressure. With the publication of the updated World Health Organization CNS tumor
classification in 2021, diagnoses have been redefined to emphasize key molecular alterations.
Gliomas represent the majority of malignant brain tumors in this age group. Glioneuronal
and neuronal tumors are associated with longstanding refractory epilepsy. The classification
of ependymomas and medulloblastomas has been refined, enabling better identification
of low-risk tumors that could benefit from treatment de-escalation strategies. Owing
to their midline location, germ cell tumors often present with oculomotor and visual
alterations as well as endocrinopathies. The management of CNS tumors in AYA is often
extrapolated from pediatric and adult guidelines, and generally consists of a combination
of surgical resection, radiation therapy, and systemic therapy. Ongoing research is
investigating multiple agents targeting molecular alterations, including isocitrate
dehydrogenase inhibitors, SHH pathway inhibitors, and BRAF inhibitors. AYA patients
with CNS tumors should be managed by multidisciplinary teams and counselled regarding
fertility preservation, psychosocial comorbidities, and risks of long-term comorbidities.
There is a need for further efforts to design clinical trials targeting CNS tumors
in the AYA population.
Keywords
brain neoplasms - glioma - adolescents and young adults
