dysembryoplastic neuroepithelial tumor
Pronunciation: dis-em-bree-oh-PLAS-tik new-roh-ep-ih-THEE-lee-al
Also written as: DNET — dysembryoplastic neuroepithelial tumor
A benign cortical tumor composed of oligodendroglia-like cells and neurons with a multinodular architecture.
Full Definition
Dysembryoplastic neuroepithelial tumor (DNET) is a benign WHO grade I cortical tumor that typically presents with medically intractable epilepsy in children and young adults. These tumors are characterized by a multinodular architecture with oligodendroglia-like cells arranged around neurons in a specific glioneuronal element pattern. DNETs show a distinctive 'floating neurons' appearance where neurons appear suspended within a mucoid matrix. The tumors are cortically based, often involve the temporal lobe, and may show calcification. Complete surgical resection is typically curative, and malignant transformation is extremely rare. The specific glioneuronal element is pathognomonic for DNET.
Usage
Usage note: Emphasize specific glioneuronal element as diagnostic feature.
In Context
- "The epilepsy surgery specimen showed dysembryoplastic neuroepithelial tumor with characteristic floating neurons." — Surgical pathology report
- "The specific glioneuronal element confirmed the diagnosis of dysembryoplastic neuroepithelial tumor." — Neuropathology consultation