胚胎发育不良性神经上皮肿瘤.ppt

CASE REPORT Dysembryoplastic Neuroepithelial Tumor (DNET) 胚胎发育不良性 神经上皮肿瘤 北京大学人民医院 陈皓 Radiological Findings CT MRI Radiologic appearance CT：A well-demarcated introcortical hypoattenuating lesion with no edema and mass effect in the right frontal-parietal lobe. MRI：The lesion shows T1WI hypointense, T2WI hyperintense, FLAIR mixed isointense with a “bright” rim, DWI isointense ,T1WI CE no enhancement. No peritumoral edema and occupying effect. MRS：No obvious change of Cho and Cr, NAA reduces slightly. Final diagnosis Dysembryoplastic Neuroepithelial Tumor （DNET） 胚胎发育不良性神经上皮瘤 Introduction Concept Dysembryoplastic teuroepithelial tumor (DNET) A rare benign intracortical supratentorial mixed neuronal-glial neoplasm. Most common located in the temporal lobe. First reported by Daumas-Duport, et al in 1988. Epidemical features Frequency: rare, 1% of all primary brain tumors. Age: adolescents and young adults within 20 years old. Gender: M≧F. Pathology A: Hallmark: Specific glioneuronal element (SGNE)=Columns of heterogenerous cells located in a neurofibrillary matrix, including: oligodendrocyte-like cells + mature ganglion cells + astrocytes. B: Typical findings: Involvement of the cortical gray matter and a multinodular growth pattern. C: Accompanying findings: The cortex adjacent to a DNET shows a disordered architecture. Left：shows the nodular growth pattern and the loose matrix of this tumor. Note the focal calcification (arrow). Right：shows many oligodendroglia-like cells with small round nuclei (arrow) and a clear perinuclear halo. These cells are located in a neurofibrillary matrix. Clinical features Partial chronic drug-resistant seizures occurred during early chilodhood. No accompanying neurologic deficits. Treatment and prognosis Drugs: to control seizures. Surgical treatment : operation to remove tumors and to cure seizures. Prognosis: free of seizures in 70