Abstract
Simple SummaryMolecular characterization of ependymoma has revolutionized its categorization. This new molecular classification has implications particularly in targeted therapeutics. Amongst the ten subgroups of ependymoma currently described, three are found in the spinal compartment, and three in the infratentorial and supratentorial compartments respectively; the subependymoma subgroup is found in all these anatomic compartments. Each subgroup carries unique molecular features that lead to oncogenesis and to disparities in prognosis. Here, the molecular classification, key clinical features, current understanding of tumorigenesis, and potential molecular targets for cranial and spinal ependymoma are discussed.Ependymoma is a biologically diverse tumor wherein molecular classification has superseded traditional histological grading based on its superior ability to characterize behavior, prognosis, and possible targeted therapies. The current, updated molecular classification of ependymoma consists of ten distinct subgroups spread evenly among the spinal, infratentorial, and supratentorial compartments, each with its own distinct clinical and molecular characteristics. In this review, the history, histopathology, standard of care, prognosis, oncogenic drivers, and hypothesized molecular targets for all subgroups of ependymoma are explored. This review emphasizes that despite the varied behavior of the ependymoma subgroups, it remains clear that research must be performed to further elucidate molecular targets for these tumors. Although not all ependymoma subgroups are oncologically aggressive, development of targeted therapies is essential, particularly for cases where surgical resection is not an option without causing significant morbidity. The development of molecular therapies must rely on building upon our current understanding of ependymoma oncogenesis, as well as cultivating transfer of knowledge based on malignancies with similar genomic alterations.
Highlights
Ependymomas are primary central nervous system (CNS) tumors derived from the ependymal lining of the ventricular system [1,2]
This observed overexpression in high-grade Supratentorial Ependymoma (STE) that independently predicts outcomes further emphasizes the importance of this molecular trait and underscores the need for further studies assessing the efficacy of programmed death ligand-1 (PD-L1) inhibitors in treating Supratentorial Ependymoma with Zinc Finger Translocation Associated (ZFTA)-Fusion (ST-ZFTA)
The novel description of the molecular origin of ependymoma has led to further understanding of this genetically diverse tumor
Summary
Ependymomas are primary central nervous system (CNS) tumors derived from the ependymal lining of the ventricular system [1,2] These tumors are found in both the pediatric and adult population and are found throughout the CNS, including the supratentorial (ST) space, the infratentorial (IT) space and the spinal (SP) compartment. Based on National Cancer Institute recommendations, all intracranial pediatric ependymoma cases require surgery and postoperative radiotherapy unless the patient is under 1 year of age [20]. EANO recommends platinum or temozolomide based on their favorable toxicity profiles, though clinical trials should be considered [11,17] Ongoing research on this topic includes locoregional delivery of chimeric antigen receptor T (CAR T) cells targeting various surface antigens expressed on recurrent ependymomas, which has been shown to be highly efficacious in a mouse model [32]. As our treatment modalities improve for this pathology, and longevity of this patient population subsequentially increases, optimization of treatment regimens for Cancers 2021, 13, 6218 recurrence using a combination of surgery, radiation, and chemo-/immuno-therapies will need to be further investigated
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