胚胎发育不良性神经上皮肿瘤
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○ 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.
CASE REPORT
Dysembryoplas tic
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
History
• M, 17 yrs • ID number: 1186959 • Intractable complex partil seizures for 10
○ A: Hallmark: Specific glioneuronal element (SGNE)=Columns of heterogenerous cells located in a neurofibrillary matrix, including: oligodendrocyte-like cells + mature ganglion cells + astrocytes.
Fig.1 shows calcification in a well-demarcated introcortical hypoattenuating tumor.
Fig.2 shows focal contrast enhancement.
yrs. • Neurological examinations: no special
companying neurologic deficit.
Radiological Findings
CT
MRI
T1WI
T2WI
FLAIR
DWI
T1WI GD-DTPA
MRS
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.
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.
Epidemical features
○ Frequency: rare, <1% of all primary brain tumors. ○ Age: adolescents and young adults within 20
years old.
○ Gender: M≧F.
Pathology
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.
Clinical features
○ Partial chronic drug-resistant seizures occurred during early chilodhood.
○ No accompanying neurologic deficits.
Treatment and prognosis
and seldom envidence of tumor recurrence and enlargement after surgery.
Radiologic features
CT:
○ Well-demarcated introcortical hypoattenuating lesion.
○ “pseudocystic” appearance. ○ Minimal or subtle calcification. (Fig. 1) ○ No or focal contrast enhancement. (Fig. 2)
○ MRS：No obvious change of Cho and Cr, NAA rቤተ መጻሕፍቲ ባይዱduces slightly.
Final diagnosis
Dysembryoplastic Neuroepithelial Tumor （DNET）
胚胎发育不良性神经上皮瘤
Introduction
Concept
○ Drugs: to control seizures. ○ Surgical treatment : operation to remove tumors
and to cure seizures. ○ Prognosis: free of seizures in 70-81% patients,
○ C: Accompanying findings: The cortex adjacent to a DNET shows a disordered architecture.
CASE REPORT
Dysembryoplas tic
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
History
• M, 17 yrs • ID number: 1186959 • Intractable complex partil seizures for 10
○ A: Hallmark: Specific glioneuronal element (SGNE)=Columns of heterogenerous cells located in a neurofibrillary matrix, including: oligodendrocyte-like cells + mature ganglion cells + astrocytes.
Fig.1 shows calcification in a well-demarcated introcortical hypoattenuating tumor.
Fig.2 shows focal contrast enhancement.
yrs. • Neurological examinations: no special
companying neurologic deficit.
Radiological Findings
CT
MRI
T1WI
T2WI
FLAIR
DWI
T1WI GD-DTPA
MRS
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.
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.
Epidemical features
○ Frequency: rare, <1% of all primary brain tumors. ○ Age: adolescents and young adults within 20
years old.
○ Gender: M≧F.
Pathology
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.
Clinical features
○ Partial chronic drug-resistant seizures occurred during early chilodhood.
○ No accompanying neurologic deficits.
Treatment and prognosis
and seldom envidence of tumor recurrence and enlargement after surgery.
Radiologic features
CT:
○ Well-demarcated introcortical hypoattenuating lesion.
○ “pseudocystic” appearance. ○ Minimal or subtle calcification. (Fig. 1) ○ No or focal contrast enhancement. (Fig. 2)
○ MRS：No obvious change of Cho and Cr, NAA rቤተ መጻሕፍቲ ባይዱduces slightly.
Final diagnosis
Dysembryoplastic Neuroepithelial Tumor （DNET）
胚胎发育不良性神经上皮瘤
Introduction
Concept
○ Drugs: to control seizures. ○ Surgical treatment : operation to remove tumors
and to cure seizures. ○ Prognosis: free of seizures in 70-81% patients,