胚胎發(fā)育不良性神經(jīng)上皮腫瘤課件

1、CASE REPORTDysembryoplastic Neuroepithelial Tumor (DNET)胚胎發(fā)育不良性神經(jīng)上皮腫瘤北京大學(xué)人民醫(yī)院 陳皓History M, 17 yrs ID number: 1186959 Intractable complex partil seizures for 10 yrs. Neurological examinations: no special companying neurologic deficit.Radiological FindingsCTMRIT1WIT2WIFLAIRDWIT1WI GD-DTPAMRSRadiologic

2、 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 an

3、d occupying effect.MRS：No obvious change of Cho and Cr, NAA reduces slightly.Final diagnosis Dysembryoplastic Neuroepithelial Tumor （DNET）胚胎發(fā)育不良性神經(jīng)上皮瘤IntroductionConceptDysembryoplastic teuroepithelial tumor (DNET)A rare benign intracortical supratentorial mixed neuronal-glial neoplasm.Most common l

4、ocated in the temporal lobe.First reported by Daumas-Duport, et al in 1988.Epidemical featuresFrequency: rare, 1% of all primary brain tumors.Age: adolescents and young adults within 20 years old.Gender: MF.PathologyA: Hallmark: Specific glioneuronal element (SGNE)=Columns of heterogenerous cells lo

5、cated 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：

6、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 d

7、rug-resistant seizures occurred during early chilodhood.No accompanying neurologic deficits.Treatment and prognosisDrugs: to control seizures.Surgical treatment : operation to remove tumors and to cure seizures.Prognosis: free of seizures in 70-81% patients, and seldom envidence of tumor recurrence

8、and enlargement after surgery.Radiologic featuresCT:Well-demarcated introcortical hypoattenuating lesion.“pseudocystic” appearance.Minimal or subtle calcification. (Fig. 1)No or focal contrast enhancement. (Fig. 2)Fig.1 shows calcification in a well-demarcated introcortical hypoattenuating tumor.Fig

9、.2 shows focal contrast enhancement.MRI:Shape of lesionMultinodular or septated “pseudocystic” appearance.(Fig.1,Fig.2)Well-demarcated,wedge-shaped bubbly configuration.Triangular pattern of distribution (triangular sign)-the tumor width that is maximal at the cortical level and decreases toward bra

10、in ventricle, may be related to the radial glial fibers pathway. (Fig. 3,Fig.4)Fig.1 shows the septated appearance.(arrow)Triangular sign: pic.B, tumor width that is maximal at the cortical level and decreases toward brain ventricle.2. Signal featuresT1WI: hypointense.T2WI: very hyperintense, multin

11、odular or septated signs.FLAIR: mixed hypo/isointense signal with “bright” rim (Fig. 1c,Fig.4), no peritumoral edema.DWI: usually lacks restricted diffusion, hypointense.T1WI enhancement: no enhancement, or focal/ring enhangcement in some tumors.(Fig.2,Fig.3)3. Circumjacent featuresNo peritumoral ed

12、ema.No occupying effect.Thick gyrus in some patients.4. MRSNo obvious change of Cho and Cr.NAA reduces slightly.Lac shows sometimes.DiagnosisClinical features: choronical Intractable seizure.Radiologic features.Pathological features.Differential diagnosisLow-grade astrocytoma（低級星形細(xì)胞瘤）Oligodendroglio

13、ma（少突膠質(zhì)瘤）Ganglioglioma（神經(jīng)節(jié)細(xì)胞膠質(zhì)瘤）Pleomorphic xanthoastrocytoma（黃色星形 細(xì)胞瘤 ） Neuroepithelial cyst（神經(jīng)上皮囊腫）Low-grade astrocytoma （低級星形細(xì)胞瘤）Occur between 2040 yrs old.Grow in deep white matter and extend along white matter tracts, cortical involvement is rare.Peritumoral edema and occupying effect.Oligodend

14、roglioma（少突膠質(zhì)瘤）Often occur in adults.Calcification along the gyrus is often seen.Frontal lobe involvement is frequent.Grow in deep white matter, and invade into adjancent cortex.Bone erosion is sometimes found.Nodular configuration and triangular sign seldom occur.Contrast enhanced.Ganglioglioma（神經(jīng)節(jié)

15、細(xì)胞膠質(zhì)瘤）Calcification is common.Change of skull.Tumor margin is usually ill-defined.Cystic or cystic/solid component.Strong contrast enhancement.Occupying effect can be seen.Ganglioglioma (a) CT shows a calcified mass (arrows) of the posteriorright temporal lobe. (b) T1WI shows a heterogeneously hypoi

16、ntense tumor with a high signal intensity (arrowheads), corresponds to the calcification seen at CT. (c) T2WI shows high signal intensity with peritumoral edema.(d) CE T1WI shows ringlike enhancementof the mass.abcdShows the thinning of the adjacent skull. (arrow) Pleomorphic xanthoastrocytoma（PXA 黃

17、色星形細(xì)胞瘤 ）Cortical and meningeal involvement.Enhancing nodule abuts pia, and “dural tail” sign. a：Contrast-enhanced CT image shows a heterogeneous mass of the right parietal region with both cyst-like and soft-tissue components and inner table remodeling in the adjacent calvaria. The mural nodule enha

18、nces homogeneously andintensely. b： Axial PD-weightedMR image shows high signal intensity of the cyst-like area in relation to CSF and isointensity of the solid component in relation to gray matter. c： Contrastenhancedsagittal T1-weightedMR image shows intense enhancement of the soft-tissue portion

19、of the mass and peripheral enhancement of the cystlikearea. a Contrast-enhanced CT image shows a heterogeneous enhancing solid mass of the left frontal lobe with some cystic changes. A moderate amount of surrounding vasogenic edema is present.b Axial T2-weighted MR image shows isointensity of the so

20、lid mass in relation to gray matter, whereas the cystic portions are hyperintense. Some “fingers” of vasogenic edema surround the mass. c Contrast enhanced axial T1-weighted MR image shows intense enhancement ofthe solid mass with rim enhancement of the cystic margin1a CT shows solid tissue with the same density as the grey matter, in the left frontallobe adjacent to the falx. 1b The lesion shows marked, homogeneous contrast enhancement.2a Axial T1-weighted image shows a slightly low-signal solid lesion that on a T2-weighted image.2b gives lower signal than the surr