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Dysembryoplastic neuroepithelial tumor (DNET)

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.

Context: Central Nervous System Tumors / Astrocytomas, Other Gliomas, and Glioneuronal/Neuronal Tumors / Molecular features of glioneuronal and neuronal tumors

DNET presents in children and adults, with the median age at diagnosis in mid-to-late adolescence. It is characterized histopathologically by the presence of columns of oligodendroglial-like cells and cortical ganglion cells floating in mucin.[61] The temporal lobe is the most common location, and it is associated with drug-refractory epilepsy.[54,62]

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FGFR1 alterations have been reported in 60% to 80% of DNETs, and include FGFR1 activating single nucleotide variants, internal tandem duplication of the kinase domain, and activating gene fusions.[4,63,64] BRAF variants are uncommon in DNET.

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Preserved source evidence · Independent clinical review pending · Not medical advice