The authors presented one case of Dysembryoplastic neuroepithelial tumor revealed at a 23 years old woman with a 6 years epileptic. The histological and immunohistochemical aspect identified a “nonspecific” DNT. The authors discussed about the clinical, CT scanner, MRI and pathologic aspects and reviewed in literature
Neuroectodermal Tumors, Primitive, Peripheral ; Neoplasms, Neuroepithelial

BACKGROUND: Perikaryal or perikaryal surface immunostaining for synaptophysin has been posited to distinguish the neoplastic neuronal elements of gangliogliomas from entrapped non-neoplastic neurons in other gliomas of various types. However, recent studies revealed that perikaryal surface immunoreactivity can be seen in the neurons of normal human spinal cords and brains, as well as in the brain tissues around certain non-neuronal lesions. To access the validity of this criterion in the diagnosis of ganglion cell neoplasms, we evaluated patterns of immunostaining of synaptophysin in neuronal, glial and some non-neuroepithelial tumors. METHODS: We selected 104 cases of gangliogliomas, gangliocytomas, central neurocytomas, dysembryoplastic neuroepithelial tumors, astrocytomas, oligodendrogliomas, glioblastomas, a pleomorphic xanthoastrocytoma, meningiomas, arterio-venous malformations, craniopharyngiomas, a foreign body granuloma, temporal lobe epilepsies, and autopsied brains. A representative block including the gray matter was identified for each case, and synaptophysin immunostaining was performed. RESULTS: Perikaryal and perikaryal surface immunoreactivity for synaptophysin was observed in the neurons of various types of lesions. Percentage of perikaryal and perikaryal surface immunoreactivity of the gangliogliomas, glial tumors, and non-neuroepithelial lesions were 100%/93%, 80%/58% and 57%/26%, respectively. CONCLUSIONS: Although synaptophysin positive neurons are found in the ganglioglioma, these patterns are clearly not pathognomonic for glioneuronal tumors.
Astrocytoma ; Brain Neoplasms* ; Brain* ; Craniopharyngioma ; Diagnosis ; Epilepsy, Temporal Lobe ; Ganglioglioma ; Ganglion Cysts ; Ganglioneuroma ; Glioblastoma ; Glioma ; Granuloma, Foreign-Body ; Humans ; Immunohistochemistry ; Meningioma ; Neoplasms, Neuroepithelial ; Neurocytoma ; Neurons ; Oligodendroglioma ; Spinal Cord ; Synaptophysin*

Mutation in the p53 suppressor gene is the most common genetic alteration found in human cancers including primary brain tumors. Ki-67 labeling index(LI) is known to be a marker of proliferating activity. The purpose of this study was to verify whether an immunohistochemical expression of p53 antibody and Ki-67 LI could be related to different clinicopathologic parameters including histologic grade, size, invasiveness and recurrence of the brain tumors. Materials were based on the 147 surgically resected brain tumors during the last two years. Of the 147 brain tumors, there were 35 astrocytic tumors, 35 meningiomas, 10 oligodendrogliomas, 7 craniopharyngiomas, 5 dysembryoplastic neuroepithelial tumors, 4 medulloblastomas, 5 ependymomas, 23 pituitary adenomas, 9 schwannomas, and 14 other brain tumors. The p53 expression and Ki-67 LI were higher in malignant brain tumors including astrocytic tumors, medulloblastoma, PNET and gliosarcoma. The p53 positivity was correlated with histologic grades and tumor recurrence. The brain tumors with a high Ki-67 LI(>6%) also showed a close relationship to a higher histologic grading, radiological invasiveness and recurrence. There was no evident correlation with the age and tumor size with p53 expression and Ki-67 LI. These results suggest that p53 overexpression and high proliferation potential of the tumor cells are associated with the higher histologic grade and aggressive clinical course in the central nervous system tumors.
Brain Neoplasms* ; Brain* ; Central Nervous System Neoplasms ; Craniopharyngioma ; Ependymoma ; Genes, Suppressor ; Gliosarcoma ; Humans ; Medulloblastoma ; Meningioma ; Neoplasms, Neuroepithelial ; Neurilemmoma ; Neuroectodermal Tumors, Primitive ; Oligodendroglioma ; Pituitary Neoplasms ; Recurrence

Despite concentrated basic and clinical research efforts including the initial successful combination of surgery, radiotherapy and chemotherapy with BCNU, significant progress in the treatment of human brain tumors have been slow and looks for more successful strategies developed based upon the information from animal model system. It is to recreate in the laboratory under experimental condition a model of human brain tumors. Although no unique model of the numerous animal tumors resembling the spontaneous human brain tumors developed in these days, experimental animal models to have own specific adventages can be induced by exposure to oncogenic viruses or chemical carcinogens. Intracerebral injection of oncorna viruses can produce glioblastoma mutiformes, astrocytomas and sarcomas, while medulloblastoma, choroids plexus papilloma and ependymomas can be induced by papova viruses, and human adenovirus may cause neuroblastoma, medulloepithelioma and retinoblastomas. Chemical induction in adult animals and transplacental chemical induction were ependymoblastomas, glioma, gliosarcoma and malignant neurinomas. Reproducibility of location, cell type, and time of tumor appearances;expense;growth in tissue culture;trauma to brain;nature of vasculature, and amount of brain and tumor tissue available for examination are the variables to be considered in choosing a model to use in evaluating drug and other therapies, cell kinetics and immunological studies.
Adenoviruses, Human ; Adult ; Animals ; Astrocytoma ; Brain Neoplasms* ; Brain* ; Carcinogens ; Carmustine ; Choroid ; Drug Therapy ; Ependymoma ; Glioblastoma ; Glioma ; Gliosarcoma ; Humans ; Kinetics ; Medulloblastoma ; Models, Animal ; Neurilemmoma ; Neuroblastoma ; Neuroectodermal Tumors, Primitive ; Oncogenic Viruses ; Papilloma ; Radiotherapy ; Retinoblastoma ; Sarcoma

The World Health Organization (WHO) classification of central nervous system (CNS) tumors was revised in 2016 with a basis on the integrated diagnosis of molecular genetics. We herein provide the guidelines for using molecular genetic tests in routine pathological practice for an accurate diagnosis and appropriate management. While astrocytomas and IDH-mutant (secondary) glioblastomas are characterized by the mutational status of IDH, TP53, and ATRX, oligodendrogliomas have a 1p/19q codeletion and mutations in IDH, CIC, FUBP1, and the promoter region of telomerase reverse transcriptase (TERTp). IDH-wildtype (primary) glioblastomas typically lack mutations in IDH, but are characterized by copy number variations of EGFR, PTEN, CDKN2A/B, PDGFRA, and NF1 as well as mutations of TERTp. High-grade pediatric gliomas differ from those of adult gliomas, consisting of mutations in H3F3A, ATRX, and DAXX, but not in IDH genes. In contrast, well-circumscribed low-grade neuroepithelial tumors in children, such as pilocytic astrocytoma, pleomorphic xanthoastrocytoma, and ganglioglioma, often have mutations or activating rearrangements in the BRAF, FGFR1, and MYB genes. Other CNS tumors, such as ependymomas, neuronal and glioneuronal tumors, embryonal tumors, meningothelial, and other mesenchymal tumors have important genetic alterations, many of which are diagnostic, prognostic, and predictive markers and therapeutic targets. Therefore, the neuropathological evaluation of brain tumors is increasingly dependent on molecular genetic tests for proper classification, prediction of biological behavior and patient management. Identifying these gene abnormalities requires cost-effective and high-throughput testing, such as next-generation sequencing. Overall, this paper reviews the global guidelines and diagnostic algorithms for molecular genetic testing of brain tumors.
Adult ; Astrocytoma ; Brain Neoplasms* ; Brain* ; Central Nervous System ; Child ; Classification ; Diagnosis ; Ependymoma ; Ganglioglioma ; Genes, myb ; Glioblastoma ; Glioma ; Humans ; Molecular Biology ; Neoplasms, Neuroepithelial ; Neurons ; Oligodendroglioma ; Promoter Regions, Genetic ; Telomerase ; World Health Organization

Cancer may be a disease of genes, arising from genetic damage of diverse sorts-recessive and dominant mutations, large rearrangement of DNA and gene translocation on chromosomes, all leading to distorisions of either the expression or biochemical function of genes. The search for these genetic damage in neoplastic cells now is the most important in cancer research. It has been found that the cancer relevant genes were located on the specific regions of chromosomes. To determine whether epidermal growth factor receptor(EGFR), P53 and bcr genes located in chromosomes 7, 17 and 22 are altered, we examined 12 neuroepithelial tumor with Southern blot analysis(five low grade astrocytoma, two high grade astrocytoma, two medulloblastoma, on oligodendroglioma, one ependymoma, one choroid plexus papilloma). The loss of heterozygosity(LOH) of EGFR gene was detected in two cases of medulloblastoma. The rearrangement of EGFR gene was detected in a case of ependymoma. The LOH of P53 gene was found in a case of choroid plexus papilloma and low grade astrocytoma. The rearrangement of P53 gene was founs id a case of oligodendroglioma. The LOH of bcr gene was observed in two cases of medulloblastoma and low grade astrocytoma. The rearrangement of bcr gene was observed in two cases of high grade astrocytoma. These results suggested that tumorigenesis and tumor development in the neuroepithelial tumor may invlove specific gene changes in chromosomes 7, 17 and 22.
Astrocytoma ; Blotting, Southern ; Carcinogenesis ; Choroid Plexus ; DNA ; Ependymoma ; Epidermal Growth Factor ; Genes, erbB-1 ; Genes, p53 ; Loss of Heterozygosity ; Medulloblastoma ; Neoplasms, Neuroepithelial* ; Oligodendroglioma ; Papilloma, Choroid Plexus

PURPOSE: To assess the metabolite ratios in gliomas to determine whether the metabolic information obtained by using by using in vivo single vexel 1H magnetic resonance spectroscopy(MRS) can be used as a marker for the grading of malignancy. MATERIALS AND METHODS: A total of 28 1H MR spectra from brain tumors in 27 patients with pathologically-proven gliomas were recorded. Seven patients had low grade gliomas (grade II astrocytoma in three, oligodendroglioma in three and mixed glioma in one), six had anaplastic gliomas (grade III astrocytoma in three and oligodendroglioma in three), and 14 had glioblastoma multiformes (grade IV), 1H MRS was performed on a 1.5T MRunit using PRESS sequence with a TR of 2000ms, a TE of 270 or 135ms and a voxel size of cm for all spectra. Relative lactate levels, NAA/Cho, NAA/Cr and Cho/Cr ratios were measured based on the peak heights of each resonance and compared among gliomas. RESULTS: Most tumors demonstrated decreased NAA, elevated Cho and lactate. Relatively high lactate and Cho levels and markedly decreased NAA level were more frequently observed in the high grade gliomas than in low grade gliomas. Marked elevation of lactate level in the solid component of the tumor was mostly observed in high grade gliomas. In a patient with gliomatosis cerebri, 1H MRS demonstrated a spectral pattern of tumor in filtration in an area that on MR images was apparently normal. However, NAA/Cr, NAA/Cho and Cho/Cr ratios did not significantly correlate, however, with the histologic grading of malignancy. Because of the partial volume effect, the heterogeneity of tumors containing solid and cystic or necrotic components within avoxel limited the interpretation of 1H MRS data for the grading of malignancy. CONCLUSION: The results suggest that in some patients in vivo single voxel 1H MRS may be useful for grading the malignancy of gliomas and evaluating the exact extent of tumors. In solid gliomas, the relative level of lactate appears to be a good markerfor the grading of malignancy.
Astrocytoma ; Brain Neoplasms ; Filtration ; Glioblastoma ; Glioma* ; Humans ; Lactic Acid ; Magnetic Resonance Spectroscopy* ; Neoplasms, Neuroepithelial ; Oligodendroglioma ; Population Characteristics

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

No abstract available.
Ganglioglioma* ; Neoplasms, Neuroepithelial ; Neuroblastoma ; Prognosis

Brain tumors are the second most common type of structural brain lesion that causes chronic epilepsy. Patients with low-grade brain tumors often experience chronic drug-resistant epilepsy starting in childhood, which led to the concept of long-term epilepsy-associated tumors (LEATs). Dysembryoplastic neuroepithelial tumor and ganglioglioma are representative LEATs and are characterized by young age of onset, frequent temporal lobe location, benign tumor biology, and chronic epilepsy. Although highly relevant in clinical epileptology, the concept of LEATs has been criticized in the neuro-oncology field. Recent genomic and molecular studies have challenged traditional views on LEATs and low-grade gliomas. Molecular studies have revealed that low-grade gliomas can largely be divided into three groups : LEATs, pediatric-type diffuse low-grade glioma (DLGG; astrocytoma and oligodendroglioma), and adult-type DLGG. There is substantial overlap between conventional LEATs and pediatric-type DLGG in regard to clinical features, histology, and molecular characteristics. LEATs and pediatric-type DLGG are characterized by mutations in BRAF, FGFR1, and MYB/MYBL1, which converge on the RAS-RAF-MAPK pathway. Gene (mutation)-centered classification of epilepsy-associated tumors could provide new insight into these heterogeneous and diverse neoplasms and may lead to novel molecular targeted therapies for epilepsy in the near future.
Age of Onset ; Astrocytoma ; Biology ; Brain ; Brain Neoplasms ; Classification ; Epilepsy ; Ganglioglioma ; Glioma ; Humans ; Molecular Targeted Therapy ; Neoplasms, Neuroepithelial ; Seizures ; Temporal Lobe