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Supratentorial ependymomas with ZFTA fusions (ST-EPN-ZFTA)
Source: Childhood Cancer Genomics (PDQ®)–Health Professional Version, National Cancer Institute.
Source updated: April 30, 2025 · Captured 2026-09-09.
Selected source text with whitespace normalised. This Triangle page is not an NCI PDQ summary. Independent clinical review is pending.
ST-EPN-ZFTA is the largest subset of pediatric supratentorial ependymomas and is predominantly characterized by gene fusions involving RELA,[181,182] a transcriptional factor important in NF-κB pathway activity. ST-EPN-ZFTA is characterized by the following:
Represents approximately 70% of supratentorial ependymomas in children,[181,182] and presents at a median age of 8 years.[166]
Presence of ZFTA fusions result from chromothripsis involving chromosome 11q13.1.[181]
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Low rates of variants that affect protein structure and near absence of recurring variants outside of ZFTA::RELA fusions.[181]
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Evidence of NF-κB pathway activation at the protein and RNA level.[181]
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Gain of chromosome 1q, in approximately one-quarter of cases, with an indeterminate effect on survival.[166]
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The concordance was high between immunohistochemistry for nuclear p65-RelA, fluorescence in situ hybridization for ZFTA and RELA, and DNA methylation-based classification for defining ST-EPN-ZFTA.[183]
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Homozygous deletion of CDKN2A has been associated with a poor prognosis in patients with ZFTA fusion–positive ependymoma.[184][Level of evidence B4] CDKN2A deletion has also been reported as a secondary event in recurrent ependymoma.[185]
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Preserved source evidence · Independent clinical review pending · Not medical advice