Supra-tentorial Ependymomas with ZFTA Fusion, YAP1 Fusion, and Astroblastomas, MN1-altered: Characteristic Imaging
Victoire Perrod1,2,3, Raphael Levy1,2,3, Arnault Tauziède-Espariat4
1Pediatric Radiology Department, AP-HP, Hôpital Universitaire Necker-Enfants Malades, 75015, Paris, France.
Clinical Neuroradiology
|August 2, 2024
Summary
MRI characteristics can help distinguish supratentorial ependymoma subtypes (ZFTA-fused, YAP1-fused) and astroblastomas. Astroblastomas show distinct frontal lobe location and higher cerebral blood flow, aiding diagnosis in pediatric brain tumors.
Area of Science:
- Neuro-oncology
- Pediatric neurosurgery
- Neuroradiology
Background:
- Supratentorial (ST) ependymomas comprise distinct molecular subgroups with differing prognoses.
- Astroblastomas, MN1-altered, present histopathologic similarities to ependymomas, posing a diagnostic challenge in children.
- Accurate differentiation is crucial for appropriate treatment and outcome prediction.
Purpose of the Study:
- To investigate if distinct MRI characteristics differentiate ZFTA-fused ependymomas, YAP1-fused ependymomas, and MN1-altered astroblastomas.
- To identify imaging features that aid in distinguishing these pediatric ST brain tumor subtypes.
Main Methods:
- Retrospective analysis of preoperative MRI scans from 45 pediatric patients with ST ependymoma or astroblastoma.
- Evaluation of imaging features including location, volume, calcifications, solid/cystic components, enhancement, and diffusion.
- Comparison of imaging characteristics across molecular subtypes: ZFTA-fused, YAP1-fused, and astroblastoma, MN1-altered.
Main Results:
- YAP1-fused ependymomas were more frequently multilobar compared to ZFTA-fused ependymomas.
- Astroblastomas predominantly occurred in the frontal lobe (100%) versus ependymomas (49%).
- Cerebral blood flow was significantly higher in astroblastomas than ependymomas, with no other significant imaging differences noted between molecular groups.
Conclusions:
- Specific MRI features, including location and cerebral blood flow, can guide the distinction between ST ependymoma subtypes and astroblastomas.
- Shared common imaging features aid in differentiating these tumors from other pediatric ST neoplasms.
