Ependymoma RELA fusion-positive
Ependymoma RELA fusion-positive is a recently accepted variant of ependymoma, only recognised in the 2016 update to the WHO classification of CNS tumours. They are the most common type of supratentorial ependymoma in children, and not found in the posterior fossa or spinal cord.
RELA fusion positive tumours account for 70% of childhood supratentorial ependymomas, and a smaller number of supratentorial tumours in adults 1.
These tumours can be both grade II or III and demonstrate a variety of histological morphologies, although clear cells and prominent vascularity are common 1. The presence of the RELA fusion gene can be assessed with FISH.
- 1. 9. Louis DN, Ohgaki H, Wiestler OD, Cavenee WK "WHO Classification of Tumours of the Central Nervous System. 4th Edition Revised" ISBN: 9789283244929