An In-Depth Analysis of Ependymoma: Neuropathological Insights, Therapeutic Strategies, and Prognostic Implications in Intracranial Ependymal Tumors

Abstract

Ependymomas are primary central nervous system (CNS) neoplasms arising from ependymal cells lining the ventricular system and spinal cord. They represent a heterogeneous group of tumors with distinct histological and molecular subtypes, making their diagnosis, management, and prognosis a complex and evolving challenge in the field of neuro-oncology. This comprehensive review delves into the intricate landscape of ependymomas, elucidating their pathogenesis, classification, and molecular underpinnings. Histologically, they can manifest as myxopapillary, subependymoma, and anaplastic ependymomas, each carrying distinct clinical implications and therapeutic strategies. Moreover, recent advancements in molecular profiling have unveiled significant genetic alterations such as amplifications in RELA, YAP1, and C11orf95-MAML2 fusion, which have profound implications for prognosis and therapeutic decision-making. We explore the intricacies of clinical management, encompassing surgical resection, radiation therapy, and chemotherapy regimens tailored to the tumor's location, grade, and molecular profile. Despite their location within the CNS, ependymomas are notorious for their diverse clinical presentations, including headache, neurological deficits, and intracranial hypertension, necessitating multidisciplinary care and vigilant surveillance. Additionally, this article investigates the latest developments in targeted therapies, immunotherapeutic approaches, and ongoing clinical trials in an attempt to provide a glimpse into the future of ependymoma treatment, as precision medicine begins to play a more significant role in guiding therapeutic decisions. Furthermore, the abstract examines the critical issue of ependymoma prognosis, emphasizing the importance of integrating histopathological and molecular data in order to stratify patients into risk categories more accurately. The role of genetic markers, such as chromosome 1q gain and chromosome 6q loss, in predicting outcomes is thoroughly explored. In summary, this article aims to offer a comprehensive perspective on ependymomas, from their cellular origins to the latest breakthroughs in their management, offering insights into their clinical course, and underscoring the critical role of multidisciplinary collaboration in advancing the understanding and treatment of these complex intracranial tumors.