This study was performed to assess the usefulness of magnetoencephalography (MEG) as a presurgical evaluation modality in Korean pediatric patients with lesional localization-related epilepsy. The medical records and MEG findings of 13 pediatric patients (6 boys and 7 girls) with localization-related epilepsy, who underwent epilepsy surgery at Seoul National University Children's Hospital, were retrospectively reviewed. The hemispheric concordance rate was 100% (13/13 patients). The lobar or regional concordance rate was 77% (10/13 patients). In most cases, the MEG spike sources were clustered in the proximity of the lesion, either at one side of the margin (nine patients) or around the lesion (one patient); clustered spike sources were distant from the lesion in one patient. Among the patients with clustered spike sources near the lesion, further extensions (three patients) and distal scatters (three patients) were also observed. MEG spike sources were well lateralized and localized even in two patients without focal epileptiform discharges in the interictal scalp electroencephalography. Ten patients (77%) achieved Engel class I postsurgical seizure outcome. It is suggested that MEG is a safe and useful presurgical evaluation modality in pediatric patients with lesion localization-related epilepsy.
Adolescent ; Brain/radionuclide imaging ; Brain Diseases/pathology ; Child ; Child, Preschool ; Epilepsies, Partial/pathology/*surgery ; Female ; Ganglioglioma/pathology ; Humans ; Infant ; Magnetic Resonance Imaging ; *Magnetoencephalography ; Male ; Malformations of Cortical Development/pathology ; Neoplasms, Neuroepithelial/pathology ; Positron-Emission Tomography ; Retrospective Studies ; Seizures/diagnosis

Dysembryoplastic neuroepithelial tumors (DNETs) arise mostly in the supratentorial cerebral cortex. A very rare case of intraventricular DNET with diffuse ependymal involvement, which causes spinal drop metastasis, is presented.
Adult ; Diagnosis, Differential ; Ependymoma/*pathology/radiotherapy ; Female ; Humans ; Lumbosacral Region/*pathology ; *Magnetic Resonance Imaging ; Neuroepithelial Cells/pathology ; Spinal Neoplasms/radiotherapy/*secondary

OBJECTIVETo study the clinicopathologic features, radiologic findings, treatment options and prognosis of dysembryoplastic neuroepithelial tumor (DNT).

METHODSThe clinicopathologic and radiologic features were retrospectively analyzed in 10 cases of DNT.

RESULTSIntractable partial seizure was the main presenting symptom in all patients. The tumor was located in temporal lobe (number = 5), frontal lobe (number = 3) or parietal lobe (number = 2). CT scan displayed a hypodense lesion. MRI scan revealed the tumor was non-enhancing T1WI hypointense and T2WI hyperintense, with internal septation and hyperintense ring around the tumor seen on FLAIR image. There was neither peritumoral edema nor mass effect. Histologically, the tumor showed the presence of glioneuronal element, with oligodendrocyte-like cells, floating neurons, astrocytes and associated microcystic changes. Immunohistochemical study demonstrated positivity for NeuN and synaptophysin in the neurons and some oligodendrocyte-like cells. Olig2 and S-100 protein were also expressed in the oligodendrocyte-like cells. Ki-67 index were lower than 1% in all cases. Nine cases were treated by complete surgical excision and the remaining case was subtotally excised. No post-operative chemotherapy or radiotherapy was given. One of the 10 cases recurred on follow up.

CONCLUSIONSCorrect diagnosis of DNT requires correlation with clinicopathologic, radiologic and immunohistochemical findings. Complete resection of the tumor and epileptogenic foci is the mainstay of treatment for DNT, with intraoperative EEG monitoring. Post-operative chemotherapy or radiotherapy is not required.

Adolescent ; Adult ; Antigens, Nuclear ; metabolism ; Basic Helix-Loop-Helix Transcription Factors ; metabolism ; Brain Neoplasms ; diagnosis ; metabolism ; pathology ; surgery ; Cerebral Cortex ; metabolism ; pathology ; Child ; Female ; Follow-Up Studies ; Humans ; Magnetic Resonance Imaging ; Male ; Neoplasms, Neuroepithelial ; diagnosis ; metabolism ; pathology ; surgery ; Nerve Tissue Proteins ; metabolism ; Neurosurgical Procedures ; Oligodendrocyte Transcription Factor 2 ; Retrospective Studies ; S100 Proteins ; metabolism ; Synaptophysin ; metabolism ; Tomography, X-Ray Computed ; Young Adult

Astrocytoma ; blood supply ; pathology ; Capillaries ; pathology ; Central Nervous System Neoplasms ; blood supply ; pathology ; Diagnosis, Differential ; Ependymoma ; blood supply ; pathology ; Glioblastoma ; blood supply ; pathology ; Hemangioblastoma ; blood supply ; pathology ; Humans ; Hyperplasia ; Microvessels ; pathology ; Neoplasms, Neuroepithelial ; blood supply ; pathology ; Oligodendroglioma ; blood supply ; pathology ; Paraganglioma ; blood supply ; pathology

Adolescent ; Adult ; Antigens, Nuclear ; metabolism ; Brain Diseases ; complications ; diagnosis ; pathology ; surgery ; Brain Neoplasms ; pathology ; Child ; Child, Preschool ; Diagnosis, Differential ; Epilepsy ; etiology ; Female ; Ganglioglioma ; pathology ; Glial Fibrillary Acidic Protein ; metabolism ; Humans ; Infant ; Magnetic Resonance Imaging ; Male ; Malformations of Cortical Development ; classification ; complications ; diagnosis ; pathology ; surgery ; Malformations of Cortical Development, Group I ; Microtubule-Associated Proteins ; metabolism ; Neoplasms, Neuroepithelial ; pathology ; Nerve Tissue Proteins ; metabolism ; Neurofilament Proteins ; metabolism ; Retrospective Studies ; Vimentin ; metabolism ; Young Adult

OBJECTIVETo study the clinicalpathologic features of intracranial multiple lesions.

METHODSThe clinical, radiologic and pathologic features of intracranial multiple lesions in 62 cases during the period from 2005 to 2009 in Xuanwu Hospital were retrospectively reviewed.

RESULTSThere were 32 males and 30 females in 62 cases. The mean age of seize onset and duration of disease were 37.4-year-old and 11.6 months, respectively. The lesions could affect cerebral hemisphere, basal ganglia, brain stem, cerebellum and other parts, most lesions were located above the tentorium. Pathological diagnosis as follows: 13 patients with glioma; metastatic tumors in 13 cases; 12 cases of central nervous system infection; immune-mediated inflammatory demyelinating disease in 8 cases; 5 cases of primary lymphoma of central nervous system; primary angiitis of the central nervous system 3 cases; mitochondrial encephalopathy 2 cases; vein thrombosis in 2 cases; Rosai-Dorfman disease in 2 cases; 2 case of radiation encephalopathy. Among them, mitochondrial encephalopathy and vein thrombosis lesions located in the cortex; metastatic tumor and blood-borne infection mainly involving junction of grey and white matter; glioma, radiation encephalopathy and demyelinating disease include white matter lesions; vascular inflammation showed cortical and subcortical white matter lesions.

CONCLUSIONSA variety of tumor and non-neoplastic diseases can be expressed in intracranial multiple lesions, which gliomas, metastatic tumor and central nervous system infections are more common. In order to improve the diagnosis of intracranial multiple lesions, active work in the brian biopsy, study the clinical, imaging and pathological findings must be closely.

Adolescent ; Adult ; Aged ; Brain Neoplasms ; diagnosis ; pathology ; surgery ; Child ; Demyelinating Diseases ; diagnosis ; pathology ; surgery ; Female ; Glioma ; diagnosis ; pathology ; surgery ; Histiocytosis, Sinus ; diagnosis ; pathology ; surgery ; Humans ; Lymphoma, Large B-Cell, Diffuse ; diagnosis ; pathology ; surgery ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Neoplasms, Neuroepithelial ; diagnosis ; pathology ; surgery ; Retrospective Studies ; Toxoplasmosis, Cerebral ; diagnosis ; pathology ; surgery ; Tuberculosis, Central Nervous System ; diagnosis ; pathology ; surgery ; Young Adult

Antigens, CD34 ; metabolism ; Astrocytoma ; metabolism ; pathology ; Carcinoma ; metabolism ; pathology ; Central Nervous System Neoplasms ; metabolism ; pathology ; Ependymoma ; metabolism ; pathology ; Fibroma ; metabolism ; pathology ; Ganglioglioma ; metabolism ; pathology ; Glial Fibrillary Acidic Protein ; metabolism ; Glioma ; metabolism ; pathology ; Gliosarcoma ; metabolism ; pathology ; Humans ; Immunohistochemistry ; Neoplasms, Neuroepithelial ; metabolism ; pathology ; Solitary Fibrous Tumors ; metabolism ; pathology

OBJECTIVETo study the immunohistochemical expression and diagnostic significance of CD34 in brain tumors of patients with refractory epilepsy.

METHODSImmunohistochemical study for CD34 was performed on formalin-fixed paraffin-embedded tissue blocks of 54 cases of brain tumors occurring in patients with refractory epilepsy. The tumor types included ganglioglioma (GG, number = 21), dysembryoplastic neuroepithelial tumor (DNT, number = 8), tumors/lesions which had the transitional features that between glioneuronal hamartia and mixed neuronal-glial tumor (number = 21) and pleomorphic xanthoastrocytoma (PXA, number = 4). Cases of glioblastoma (number = 4) and oligoastrocytoma (number = 5) were used as controls.

RESULTSTwenty of the 21 cases of GG, 1 of the 8 cases of DNT, 16 of the 21 cases of tumors/lesions which had the transitional features and 3 of the 4 cases of PXA showed cytoplasmic and membranous positivity for CD34. The adjoining brain tissues in 9 of the 18 cases of GG, 6 of the 16 cases of tumors/lesions which had the transitional features and 1 of the 3 cases of PXA also expressed CD34. In contrast, only 1 case of glioblastoma showed membranous positivity for CD34.

CONCLUSIONSCD34 preferred to staining for GG and PXA. Which represent a valuable tool for distinguishing GG, PXA and DNT, oligoastrocytoma, glioblastoma.

Antigens, CD34 ; metabolism ; Astrocytoma ; complications ; metabolism ; pathology ; surgery ; Brain Neoplasms ; complications ; metabolism ; pathology ; surgery ; Diagnosis, Differential ; Epilepsy ; etiology ; Ganglioglioma ; complications ; metabolism ; pathology ; surgery ; Glioblastoma ; complications ; metabolism ; pathology ; Humans ; Neoplasms, Neuroepithelial ; complications ; metabolism ; pathology ; surgery

BACKGROUNDDysembryoplastic neuroepithelial tumor (DNT) is a rare benign neoplasm of the central nervous system affecting young people. A correct preoperative diagnosis is helpful for planning surgical strategies and improving prognosis. The purpose of this study was to characterize DNTs using magnetic resonance imaging (MRI) and magnetic resonance spectroscopy (MRS) and to analyze the value of these two techniques in the diagnosis of DNTs.

METHODSMR images of 13 patients with DNTs were reviewed retrospectively; and five of the patients also underwent MRS. Tumors were confirmed by surgery. The distribution, extension and signal features of the lesions were assessed, and the MRS results were analyzed.

RESULTSAll tumors were supratentorial. The cortex was the main area involved, with nine tumors located in the temporal lobe, three in the frontal lobe, and one on the boundary between the temporal and occipital lobes. All cases had decreased signal intensity on T1-weighted MR images and increased signal intensity on T2-weighted images. On fluid attenuated inversion recovery weighted images, the hyperintense "ring sign" and internal septation of the lesion were seen in 9 cases. Eight tumors had well-demarcated borders. Peritumoral edema or mass effect was absent in all cases. A contrast enhancement examination was performed in 9 cases. Contrast enhancement was absent in five cases, and four cases showed significant enhancement. The MRS showed a low N-acetylaspartate peak and a lack of elevated choline-containing component (Cho) or Cho-Cr ratio (Cho/Cr) in five patients.

CONCLUSIONSThe MRI findings of DNTs were stereotypical. The combination of MRI and MRS techniques were helpful in making a correct presurgical diagnosis.

Adolescent ; Adult ; Brain Neoplasms ; pathology ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Magnetic Resonance Imaging ; methods ; Magnetic Resonance Spectroscopy ; methods ; Male ; Neoplasms, Neuroepithelial ; pathology ; Retrospective Studies ; Young Adult

OBJECTIVETo study the clinicopathologic features, immunophenotype and histogenesis of dysembryoplastic neuroepithelial tumor (DNT).

METHODSFourteen cases of DNT were retrieved from the archival files of the Department. The histopathologic features and immunohistochemical findings were retrospectively studied. The long-term follow-up data were analyzed.

RESULTSEleven of the 14 cases studied were located in the temporal lobe. Histologically, the tumor consisted of a heterogeneous admixture of neuronal and glial cells (including 1 simple form case, 8 complex form cases and 5 non-specific form cases). The specific glioneuronal element was seen in 9 cases. Variable degrees of cortical dysplasia (CD) were found in 10 out of the 11 cases which had sufficient tissue samples for thorough histologic examination. The morphologic appearance of CD included the presence of heterotopic neurons in molecular layer and/or white matter (7 cases), persistent subpial granular cell layer (4 cases), dyslamination (10 cases) and cellular abnormalities. Immunohistochemically, the oligodendroglial-like cells expressed Olig2. Some of which were positive for nestin, MAP-2, neurofilament and glial fibrillary acidic protein, but negative for NeuN. Long-term follow up revealed that 12 patients had class I postoperative seizure and 2 patients had class II seizure. No tumor recurrence was detected.

CONCLUSIONSDNT is frequently associated with CD. The morphologic diagnosis can be confirmed by immunohistochemical study using a panel of antibodies.

Adolescent ; Adult ; Anticonvulsants ; therapeutic use ; Basic Helix-Loop-Helix Transcription Factors ; metabolism ; Brain Neoplasms ; complications ; metabolism ; pathology ; surgery ; Child ; Child, Preschool ; Epilepsy ; drug therapy ; etiology ; Female ; Follow-Up Studies ; Humans ; Infant ; Intermediate Filament Proteins ; metabolism ; Male ; Malformations of Cortical Development ; complications ; pathology ; Microtubule-Associated Proteins ; metabolism ; Neoplasms, Neuroepithelial ; complications ; metabolism ; pathology ; surgery ; Nerve Tissue Proteins ; metabolism ; Nestin ; Oligodendrocyte Transcription Factor 2 ; Oligodendroglia ; pathology ; Retrospective Studies ; Young Adult