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  • Updated 03.18.2023
  • Released 02.16.1999
  • Expires For CME 03.18.2026

Dysembryoplastic neuroepithelial tumor



Dysembryoplastic neuroepithelial tumors are rare, indolent, low-grade glioneuronal tumors diagnosed predominantly in children and young adults. Most commonly affecting the temporal lobe and its mesial structures, these tumors classically present with seizures before the age of 20 years. Refractory epilepsy is often managed and cured by complete surgical resection of the tumor. Pathology shows glioneuronal elements and so-called floating neurons. Dysembryoplastic neuroepithelial tumors frequently harbor FGFR1 mutations (and not fusions) that ultimately lead to RAS/MAPK pathway activation.

Key points

• Dysembryoplastic neuroepithelial tumor is an indolent WHO grade I glioneuronal tumor usually diagnosed in children and young adults.

• This supratentorial tumor most commonly occurs in the temporal lobe and presents with focal seizures.

• Imaging classically shows a (multi)cystic, lobulated, well-circumscribed tumor with expansion of a cortical gyrus.

• Pathology findings are characterized by glioneuronal elements and floating neurons, whereas molecular analysis often reveals an activating mutation of FGFR1.

• Although these tumors are considered benign, surgical resection is recommended for refractory epilepsy.

• Prognosis for seizure control following resection is excellent.

Historical note and terminology

Dysembryoplastic neuroepithelial tumor is a rare but well-recognized tumor of children and young adults that most often results in chronic epilepsy. In 1988, the clinicopathological features were first elucidated by Daumas-Duport and colleagues, who found tumors in 20 patients undergoing epilepsy surgery at St. Anne Hospital in Paris for which existing tumor classification seemed inadequate. These multinodular tumors, composed of both glial and neuronal elements, were remarkably similar to each other and resulted in a distinct clinical picture. Review of the Mayo Clinic Tissue Registry identified 19 additional cases, and the original report of 39 patients remains one of the most thorough and authoritative accounts of this tumor (10). Several other patient series have confirmed the findings of the original report (46; 55; 43), and further study has expanded the histological and molecular recognition and understanding of this tumor (09; 11; 19; 47; 53). Our knowledge of dysembryoplastic neuroepithelial tumor continues to expand due to a growing body of genomic information.

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