Dysembryoplastic Neuroepithelial Tumor
The Common vein Copyright 2010
Definition
Dysembryoplastic neuroepithelial tumor is a rare low grade mixed neuronal-glial tumor that arises from within the cortex or deep gray matter of young adults and children. It is most often found in the superficial temporal lobe and presents with seizures.
Pathologically these tumors are made of several cell types including a discrete mixture of oligodendrocyte-like cells and mature ganglion and astrocytes in a myxoid matrix. Surrounding these nodules of mixed cells types there may be regions of cortical dysplasia or even low grade astrocytoma.
Patients with these tumors present with seizures. Focal neurologic defects are unusual.
Diagnosis can be suspected on clinical history in combination with imaging findings, however definitive diagnosis is made by pathology.
Both CT and MRI can be useful to visualize theses tumors. They can appear as regions of hypoattenuation on CT. Occasionally, calcifications can be identified. On MRI they are T1 hypointense and T2 hyperintense cortically based lesions often described as a having a “soap bubble” appearance. Enhancement is not a dominant feature of DNETs but it may be seen. Often there may be bony remodeling of the skull overlying the lesion.
Treatment for dysembryoplastic neuroepithelial tumor is surgical resection. The prognosis for these patients is excellent.
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Robbins
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Robbins