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. 

Epidemiology

RELA fusion positive tumours account for 70% of childhood supratentorial ependymomas, and a smaller number of supratentorial tumours in adults 1.

Pathology

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. 

Immunophenotype
  • GFAP positive
  • EMA positive
  • L1CAM positive 1
    • correlates closely with the presence of RELA fusion
    • not exclusive to ependymomas
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Article Information

rID: 46047
Synonyms or Alternate Spellings:

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