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  • Updated 03.22.2021
  • Released 02.16.1999
  • Expires For CME 03.22.2024

Dysembryoplastic neuroepithelial tumor

Introduction

Overview

Dysembryoplastic neuroepithelial tumors are rare, indolent, low-grade tumors found most often in children and young adults. Most commonly affecting the temporal lobe, these tumors classically present with seizures. Refractory epilepsy is often managed and cured by surgical resection of the tumor.

Key points

• Dysembryoplastic neuroepithelial tumor is a highly indolent (WHO grade I) glial tumor usually found in children and young adults.

• It most commonly occurs in the temporal lobe but rarely can occur in other locations. The vast majority of tumors present with epilepsy.

• Imaging classically shows a cystic, nonenhancing tumor with expansion of a cortical gyrus.

• Although 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 pathology and clinical syndrome 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 (08). 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 the most thorough and authoritative account of this tumor to date (08). Several other patient series have confirmed the findings of the original report (33; 39; 31), and further study has expanded the pathologic recognition and understanding of this tumor (07; 09; 17). Though it remains uncommon, this tumor type is well recognized.

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