# In vitro and in vivo modeling systems of supratentorial ependymomas

## TL;DR

This paper reviews in vitro and in vivo models of supratentorial ependymomas to better understand and treat these rare brain tumors.

## Contribution

The paper provides a comprehensive review of modeling systems for supratentorial ependymomas, highlighting their strengths and limitations.

## Key findings

- Supratentorial ependymomas are characterized by gene fusions like ZFTA and YAP1.
- High tumor heterogeneity and limited treatment options contribute to poor outcomes in some ST-EPN variants.
- Developing preclinical models is crucial for testing new therapies due to the rarity of patient samples.

## Abstract

Ependymomas are rare brain tumors that can occur in both children and adults. Subdivided by the tumors’ initial location, ependymomas develop in the central nervous system in the supratentorial or infratentorial/posterior fossa region, or the spinal cord. Supratentorial ependymomas (ST-EPNs) are predominantly characterized by common driver gene fusions such as ZFTA and YAP1 fusions. Some variants of ST-EPNs carry a high overall survival rate. In poorly responding ST-EPN variants, high levels of inter- and intratumoral heterogeneity, limited therapeutic strategies, and tumor recurrence are among the reasons for poor patient outcomes with other ST-EPN subtypes. Thus, modeling these molecular profiles is key in further studying tumorigenesis. Due to the scarcity of patient samples, the development of preclinical in vitro and in vivo models that recapitulate patient tumors is imperative when testing therapeutic approaches for this rare cancer. In this review, we will survey ST-EPN modeling systems, addressing the strengths and limitations, application for therapeutic targeting, and current literature findings.