Dysembryoplastic neuroepithelial tumor

Andrea Wasilewski MD (

Dr. Wasilewski of the University of Rochester has no relevant financial relationships to disclose.

)
Rimas V Lukas MD, editor. (

Dr. Lukas of Northwestern University Feinberg School of Medicine received honorariums from AbbVie as a guest speaker, from NewLink Genetics and Reneuron for consulting work, and from Monetris as an advisory board member.

)
Originally released February 16, 1999; last updated April 6, 2019; expires April 6, 2022

This article includes discussion of dysembryoplastic neuroepithelial tumor, DNT, and DNET. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

Dysembryoplastic neuroepithelial tumors are rare, indolent, low-grade tumors found in children and young adults. Most commonly affecting the temporal lobe, these tumors often 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 (Daumas-Duport et al 1988). 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 (Daumas-Duport et al 1988). Several other patient series have confirmed the findings of the original report (Raymond et al 1994; Taraturo et al 1995; Prayson et al 1996), and further study has expanded the pathologic recognition and understanding of this tumor (Daumas-Duport 1993; Daumas-Duport et al 1999; Honavar et al 1999). Though it remains uncommon, with just over 300 cases reported fully in the literature to date, this tumor is well recognized.

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