Dysembryoplastic neuroepithelial tumor

Scott Mintzer MD (Dr. Mintzer of Thomas Jefferson University has no relevant financial relationships to disclose.)
Edward J Dropcho MD, editor. (Dr. Dropcho of Indiana University Medical Center has no relevant financial relationships to disclose.)
Originally released February 16, 1999; last updated November 14, 2014; expires November 14, 2014

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

Key points

 

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

 

• It is most commonly found in the temporal lobe and occasionally in frontal or other locations. The vast majority of tumors present with chronic epilepsy.

 

• Tumors appear on imaging as an expansion of a cortical gyrus, usually nonenhancing.

 

• Treatment is exclusively by resection, if necessary for seizure control.

 

• 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. In retrospect, these tumors resembled "certain small tumours encountered in the temporal lobe" by Cavanagh 30 years earlier, although the tumors described by Cavanagh were microscopic in scale and were considered by the author to be hamartomatous in nature (Cavanagh 1958). 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 has gained increasing recognition and is now well-known to neurologists in 2 subspecialties, as these patients are more likely to present to the refractory epilepsy clinic than to the neuro-oncology clinic.

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