Brain Neoplasms ; pathology ; Cerebellar Neoplasms ; pathology ; Ganglioglioma ; pathology ; Ganglioneuroma ; pathology ; Humans ; Neoplasms, Neuroepithelial ; pathology

A case of diffuse cerebrospinal gliomatosis with extensive leptomeningeal spread is presented. The patient, an 18-year-old girl, was admitted due to progressive weakness and paresthesia of both legs, following rapid neuropsychiatric deterioration. An initial magnetic resonance imaging (MRI) study of the T-spine showed diffuse high signal intensities from T9 to T12 spinal cords on a T2 sagittal image and diffuse cord bulging at T1WI. This suggested an inflammatory lesion such as tuberculosis or fungal meningoencephalitis. A limited autopsy was performed. A microscopic examination revealed multifocal GFAP-positive astrocytic proliferations that were low grade astrocytoma in the cerebral leptomeninges, parietal, occipital and temporal lobes and anaplastic astrocytoma in the spinal cord and spinal leptomeninges. The high proliferative indices of the spinal lesion and aneuploidy correspond to a diagnosis of malignant astrocytoma and a rapid fatal clinical course.
Adolescence ; Brain/pathology ; Case Report ; Cell Division ; Diagnosis, Differential ; Female ; Human ; Magnetic Resonance Imaging ; Meninges/pathology* ; Neoplasms, Neuroepithelial/pathology* ; Neoplasms, Neuroepithelial/diagnosis ; Spinal Cord/pathology

Cortical dysplasia (CD) is considered to be a malformative lesion of the neocortex which exhibits a spectrum of pathologic changes reflecting a disturbance in the process of its development. CD is recently recognized as a major cause of intractable epilepsy with non-neoplastic lesions. Mischel et al. proposed that CD can be graded mild, moderate and severe with regard to nine specific microscopic abnormalities: mild CD consists of 1) cortical laminar disorganization, 2) single heterotopic white matter neurons, 3) neurons in the cortical molecular layer, 4) persistent remnants of the subpial granular cell layer, and 5) marginal glioneuronal heterotopia; moderate CD displays 6) polymicrogyria and 7) white matter neuronal heterotopia; severe CD phows 8) neuronal cytomegaly with associated cytoskeletal abnormalities and 9) balloon cell change. We reassessed 71 cases of cortical dysplasia to elucidate the proportion and histologic features of each group, using Mischel's grading system. CD was most frequently found in the temporal lobe with 50 cases (70%). Mild CD was predominently seen and was noted in 61 cases (86%) Cortical laminar disorganization and single heterotopic white matter neurons were identified in all mild CD cases. Neurons in the cortical molecular layer, persistent subpial granular cell layer, and marginal glioneuronal heterotopia were also noted in case numbers 40, 3, and 1 of mild CD, respectively. Moderate CD was composed of 2 cases with polymicrogyria, and the remaining 8 cases had severe CD. All moderate and severe CD were associated with the various histological features of mild CD. Thirty eight cases (51%) of CD showed dual pathology, composed of both CD and hippocampal sclerosis, and 5 cases of dysembryoplastic neuroepithelial tumor also had CD. Neurofilament immunostain revealed disarray of abnormally beaded axons in CD. We believe that the grading system of CD is very important to the evaluation and classification of CD.
Axons ; Classification ; Epilepsy ; Malformations of Cortical Development* ; Neocortex ; Neoplasms, Neuroepithelial ; Neurons ; Pathology ; Sclerosis ; Temporal Lobe

Adult ; Brain Neoplasms ; diagnosis ; metabolism ; pathology ; Female ; Humans ; Magnetic Resonance Imaging ; Magnetic Resonance Spectroscopy ; Neoplasms, Neuroepithelial ; diagnosis ; metabolism ; pathology

OBJECTIVE: The spinal cord tumors(including vertebral tumors) are increasingly diagnosed and operated due to development of refined diagnostic and therapeutic tools. It is necessary to re-evaluate clinical features and surgical results of spinal cord tumors with increasing cases and developing treatment modalities. The authors reviewed the spinal cord tumor cases to evaluate their clinical characteristics. MATERIALS AND METHODS: The retrospective review of 654 cases of spinal cord tumors between 1973 and 1999 was done. The clinical features, pathological analysis and surgical results were analyzed and compared to the literature. The results of the study are analyzed with a more detailed consideration of each of major pathologies: neurogenic tumors, meningeal tumors, neuroepithelial tumors, and metastatic tumors. RESULTS AND CONCLUSION: The spinal cord tumor was most common in the 5th decade of age(145 cases, 22.1%) and 78 cases(11.9%) were found in children under 15 years of age. The ratio of male to female was 1.2:1. The pathologic diagnosis was neurogenic tumor in 266 cases(40.7%), neuroepithelial tumor in 131(20.0%), metastatic tumor in 118(18.0%), and meningeal tumor in 94(14.4%) in the order of frequency. The tumor was located most frequently in the thoracic area(36.5%) and in the intradural extramedullary space(38.1%). The most common initial presentation was pain(40.1%) and the mean duration for presentation to operation was 14.8 months. The total or gross total removal was possible in 404 cases(61.7%) and the surgical result on the postoperative one month was recovery or improvement in 424 cases(64.8%), stationary in 188(28.7%), progression in 42(6.4%). As a surgical complication, there was a spinal deformity(12 cases), wound infection(5 cases), aspiration pneumonia(5 cases) etc. Neurogenic tumors and menigiomas showed good surgical results, whereas neuroepithelial tumors(except ependymoma) and metastatic tumors showed relatively poor prognosis.
Child ; Diagnosis ; Female ; Humans ; Male ; Meningeal Neoplasms ; Neoplasms, Neuroepithelial ; Pathology ; Prognosis ; Retrospective Studies ; Spinal Cord Neoplasms* ; Spinal Cord* ; Wounds and Injuries

Adult ; Brain ; pathology ; Brain Neoplasms ; metabolism ; pathology ; surgery ; Diagnosis, Differential ; Female ; Ganglioglioma ; metabolism ; pathology ; surgery ; Humans ; Neoplasms, Neuroepithelial ; pathology ; S100 Proteins ; metabolism ; Synaptophysin ; metabolism

BACKGROUNDS: A dysembryoplastic neuroepithelial tumor (DNT) is an important cause of the intractable epilepsy. The prognosis after epileptic surgery was excellent when the epileptogenic zones were completely excised. But, the exact epileptogenic zones in epilepsy patients with DNT are not well known. The purpose of this study was to investigate where the epileptogenic zones were in epilepsy patients with DNT. That results might be helpful in having good results of epileptic surgery of DNT. METHODS: Six patients with medically determined intractable epilepsy due to DNT were studied retrospectively. They had undergone epileptic surgery after video-EEG monitoring not only with scalp electrodes but also with invasive subdural electrodes. DNTs were located in the temporal lobe and pathologically proven in all patients. We analyzed distributions of ictal onset zones and irritative zones with non-invasive and invasive Video-EEG monitoring. Also, we reviewed the clinical features, neuroimaging features, neuropsychological tests, Wada tests and pathological findings. The operative strategy included complete resection of DNT, ictal onset and irritative zones in all patients. In two patients, modified operative strategy including amygdalohippocampectomy was done due to mesial involvement. A modified Engel's classification was used to determine surgical outcomes. RESULTS: The irritative zones (included only more than 10% of total interictal discharges during the monitoring) showed various patterns from multifocal to unobserved. Ictal onset zones were multiple in 5 patients (dual: 3 patients, triple: 2 patients). In 1 patient, contralateral hemispheric mirror focus was observed. Mesial temporal involvement was seen in 2 patients from EEG and in 1 patient from pathology. There were discrepancies between ictal onset zones and irritative zones in 4 patients. The EEG seizures without clinical events were recorded in 1 patient. Postoperatively 5 patients were free of seizure, 1 patient had rare seizures. CONCLUSION: Not only the wide or multiple distribution of epileptogenic zones around or in the lesion but also dual pathology of hippocampus are possible in epilepsy patients with DNT. The operative strategy including the epileptogenic zones can yield a good surgical outcome.
Classification ; Electrodes ; Electroencephalography ; Epilepsy* ; Hippocampus ; Humans ; Neoplasms, Neuroepithelial* ; Neuroimaging ; Neuropsychological Tests ; Pathology ; Prognosis ; Retrospective Studies ; Scalp ; Seizures ; Temporal Lobe

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

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 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