Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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September 3, 2012
Dysembryoplastic Neuroepithelial Tumor (DNET)
- DNET is a benign mixed glial-neuronal cortical neoplasm of children and young adults with a long history of partial seizures. It is an intracortical mass superimposed on background of cortical dysplasia. They are reported in 5-80% of epilepsy specimens.
- Neurologic symptoms include partial complex seizures refractory to Rx.
- Key Diagnostic Features: Well-demarcated, "bubbly" intracortical mass is often seen with minimal or no mass effect. The tumor is essentially slow growing and can cause bone remodeling. The temporal lobe is the most common site. The tumor typically appears hypointense on T1WI, hyperintense on T2WI, and does not exhibit any enhancement. Spectroscopy study is non-specific, although lactate peak may be present. CBV map shows the absence of increase vascularization in the mass.
- DDx: Taylor cortical dysplasia, neuroepithelial cyst, ganglioglioma, and pleomorphic xanthoastrocytoma.
- Rx: surgical resection
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