Choroid Plexus Neoplasms ; pathology ; Female ; Humans ; Infant ; Lateral Ventricles ; pathology

Angiomatosis ; complications ; pathology ; Brain ; blood supply ; Brain Diseases ; complications ; pathology ; Brain Neoplasms ; complications ; pathology ; Epilepsy ; etiology ; pathology ; surgery ; Ganglioglioma ; complications ; pathology ; Humans ; Malformations of Cortical Development ; classification ; complications ; pathology ; Meninges ; blood supply

Astrocytoma ; genetics ; metabolism ; pathology ; Brain Neoplasms ; classification ; genetics ; metabolism ; pathology ; Child ; Child, Preschool ; Chromosomal Proteins, Non-Histone ; genetics ; metabolism ; Chromosome Deletion ; Cyclin-Dependent Kinase Inhibitor p16 ; genetics ; metabolism ; DNA-Binding Proteins ; genetics ; metabolism ; Ependymoma ; genetics ; metabolism ; pathology ; Hedgehog Proteins ; genetics ; metabolism ; Humans ; Medulloblastoma ; classification ; genetics ; metabolism ; pathology ; Proto-Oncogene Proteins B-raf ; genetics ; metabolism ; Rhabdoid Tumor ; genetics ; metabolism ; pathology ; SMARCB1 Protein ; Signal Transduction ; Transcription Factors ; genetics ; metabolism ; Wnt Proteins ; metabolism ; beta Catenin ; genetics ; metabolism

Adenocarcinoma ; genetics ; metabolism ; Angiomyolipoma ; etiology ; Antibiotics, Antineoplastic ; therapeutic use ; Astrocytoma ; etiology ; Brain Neoplasms ; etiology ; Breast Neoplasms ; genetics ; metabolism ; Gene Expression Regulation, Neoplastic ; Genes, Tumor Suppressor ; Humans ; Kidney Neoplasms ; etiology ; Lung Neoplasms ; genetics ; metabolism ; Mutation ; Sirolimus ; therapeutic use ; TOR Serine-Threonine Kinases ; antagonists & inhibitors ; metabolism ; Tuberous Sclerosis ; complications ; drug therapy ; genetics ; metabolism ; Tumor Suppressor Proteins ; genetics ; metabolism

Objective To quantify the neurons in the temporal lobe white matter and find their distribution in the neurologically normal individuals.Methods The temporal lobe at the level of exterior geniculata body from brain autopsy samples of 14 neurologically normal individuals were made into large slice followed by quantitative analysis of neuron density,cell density,ratio and diameter of the neuronal nuclear and the distribution of white matter neurons using two-dimensional cell counting methods.Results With the depth of the white matter of the temporal lobe increasing,the neuron density decreased from 29.26 neurons/ mm~2 to 7.32 neurons/mm~2 and 0.00 neurons/mm~2,respectively;the cell density,neuron ratio and diameter of the neuronal nuclei all decreased.Conclusion There are neurons in the temporal lobe white matter of neurologically normal individuals,whose distribution of neurons is related to the depth of white matter.

OBJECTIVETo investigate the diagnostic significance of D2-40 and annexin-1 in the ependymal tumors.

METHODSTo analyses the expression of D2-40, annexin-1, EMA and Ki-67 by immunohistochemistry in 52 cases of ependymal tumors (48 cases of ependymomas, 4 cases of choroid plexus papilloma) from Xuanwu Hospital from 2005 to 2009. Ten cases of corresponding normal brain tissue were also obtained as control.

RESULTSThirty-two of forty-eight (66.7%) cases of ependymomas were positive for D2-40. "Dot-like" and "ring-like" structures were commonly observed in ependymomas (55.3%, 21 of 38 cases) and anaplastic ependymomas (5 of 6 cases) with D2-40 staining. There was no difference in the expression between D2-40 and Ki-67 (r(s) = -0.013, P = 0.931). For annexin-1, 87.5% (42 of 48 cases) of the ependymomas were positive. The specific "granular structures" and cilium were observed in ependymomas (1 of 4 cases of myxopapillary ependymomas and 11 of 38 cases of ependymomas respectively) for annexin-1. The difference in expression between annexin-1 and Ki-67 was statistically significant (r(s) = -0.405, P = 0.005). D2-40 in combination of EMA and annexin-1 increased the positive rate to 100% in ependymomas. Choroid plexus papillomas were all positive for D2-40 and annexin-1. The control tissue was negative for D2-40 but positive for annexin-1 in the capillaries.

CONCLUSIONSThe specific structures are valuable in diagnosing of ependymal-genetic tumors, and are highlighted by D2-40 and annexin-1. D2-40 in combination of EMA and annexin-1 is a useful diagnostic marker for ependymal tumors.

Adolescent ; Adult ; Annexin A1 ; metabolism ; Biomarkers, Tumor ; metabolism ; Brain Neoplasms ; diagnosis ; metabolism ; pathology ; Child ; Child, Preschool ; Ependymoma ; diagnosis ; metabolism ; pathology ; Female ; Humans ; Immunohistochemistry ; Ki-67 Antigen ; metabolism ; Male ; Membrane Glycoproteins ; metabolism ; Middle Aged ; Mucin-1 ; metabolism ; Papilloma, Choroid Plexus ; diagnosis ; metabolism ; pathology ; Young Adult

OBJECTIVETo study the expression of P-glycoprotein, multi-drug resistance associated protein and major vault protein in pathologic brain specimens, and to investigate their roles in the pathogenesis of refractory epilepsy.

METHODSImmunohistochemical study was performed in pathology specimens from 18 cases of refractory epilepsy (including 5 cases of focal cortical dysplasia, 3 cases of tuberous sclerosis, 5 cases of ganglioglioma and 5 cases of dysembryoplastic neuroepithelial tumor).

RESULTSBoth the P-glycoprotein and major vault protein were localized in microvascular endothelium of the lesions. Major vault protein was also seen in balloon cells and some neuronal cells. On the other hand, multi-drug resistance associated protein was mainly localized in the neuronal component of the lesions. In general, the expression of P-glycoprotein and major vault protein in tumoral tissue was higher than that in non-tumoral tissue. The expression of multi-drug resistance associated protein and major vault protein was also different in the neoplastic glial cells of ganglioglioma and dysembryoplastic neuroepithelial tumor.

CONCLUSIONSP-glycoprotein, multi-drug resistance associated protein and major vault protein contribute to the pathogenesis of refractory epilepsy. They may however have different roles, with different cellular localization.

ATP-Binding Cassette, Sub-Family B, Member 1 ; analysis ; Adolescent ; Adult ; Brain ; metabolism ; Brain Neoplasms ; chemistry ; Child ; Drug Resistance, Multiple ; Epilepsy ; drug therapy ; genetics ; metabolism ; Female ; Ganglioglioma ; genetics ; metabolism ; Humans ; Male ; Malformations of Cortical Development ; genetics ; metabolism ; Multidrug Resistance-Associated Proteins ; genetics ; metabolism ; pharmacology ; Tuberous Sclerosis ; metabolism ; Young Adult

OBJECTIVETo evaluate the alteration of subunits composition in NMDA receptor and the alterations of the expression and distribution of NMDA receptors and parvalbumin (PV)-positive neurons in focal cortical dysplasia (FCD) cortices.

METHODSTwenty cases of FCD samples (including all four subtypes of FCD) obtained during epilepsy surgery and 4 controls were analysed by immunohistochemical staining for NR1, NR2A/B and PV.

RESULTSIncreased expression of NR1 was detected in the giant neurons and dysmorphic neurons in FCD; while pronounced expression of NR2A/B was detected in immature neurons, giant neurons and dysmorphic neurons of FCD, especially in somata and processes of the immature neurons. Compared with the controls, FCD cortices showed prominent scattered arrangement of PV positive neurons and fibers, dramatically decreased number of PV positive interneurons and PV background staining, especially in foci of FCD II subtype.

CONCLUSIONThere are increased expressions of NR1 and NR2A/B subunits in FCD abnormal neurons, as well as scattered and reduced expressions of PV positive neurons and fibers in FCD cortices.

Adolescent ; Biomarkers, Tumor ; metabolism ; Cerebral Cortex ; metabolism ; pathology ; Child ; Child, Preschool ; Epilepsies, Partial ; pathology ; Female ; Gene Expression Regulation ; Humans ; Infant ; Interneurons ; metabolism ; Male ; Malformations of Cortical Development ; pathology ; Neurons ; metabolism ; Parvalbumins ; analysis ; isolation & purification ; Receptors, N-Methyl-D-Aspartate ; genetics ; metabolism

OBJECTIVETo study the clinicopathologic features of brain tumors occurring in patients with medically intractable epilepsy.

METHODSThe clinical, radiologic and pathologic features of brain tumors occurring in 35 patients with intractable epilepsy encountered during the period from January, 2005 to April, 2008 in Xuanwu Hospital were retrospectively reviewed.

RESULTSThe mean age of seizure onset and duration of disease were 14.3-year-old and 8.6 years, respectively. Abnormal signals were observed in 94.3% of cases (33/35) by magnetic resonance imaging. The histologic types of brain tumors included ganglioglioma (13/35, WHO grade I and 6/35, WHO grade II), dysembryoplastic neuroepithelial tumor (3/35, WHO grade I), pleomorphic xanthoastrocytoma (3/35, WHO grade II), diffuse astrocytoma (1/35, WHO grade II), oligoastrocytoma (1/35, WHO grade II), angiocentric glioma (1/35, WHO grade I) and meningioangiomatosis (1/35). The 6 remaining cases showed features seen in between glioneuronal hamartoma and mixed neuronal-glial tumor. Most of these tumors were located in the temporal lobe (27/35) and associated with focal cortical dysplasia. Immunohistochemical study showed a remarkable expression of CD34 in gangliogliomas.

CONCLUSIONSBrain tumors in patients with medically intractable epilepsy are almost always benign and located in the temporal lobe. Most of them represent mixed neuronal-glial tumors and some show transitional features in-between glioneuronal hamartoma and mixed neuronal-glial neoplasm. The similar morphologic pattern and biological behavior of glioneuronal hamartoma and mixed neuronal-glial tumor may suggest a common pathogenetic mechanism.

Adolescent ; Adult ; Antigens, CD34 ; metabolism ; Astrocytoma ; complications ; metabolism ; pathology ; Brain Diseases ; complications ; metabolism ; pathology ; Brain Neoplasms ; complications ; metabolism ; pathology ; Child ; Child, Preschool ; Epilepsy ; etiology ; metabolism ; Female ; Ganglioglioma ; complications ; metabolism ; pathology ; Glioma ; complications ; metabolism ; pathology ; Hamartoma ; complications ; metabolism ; pathology ; Humans ; Infant ; Magnetic Resonance Imaging ; Male ; Oligodendroglioma ; complications ; metabolism ; pathology ; Retrospective Studies ; Temporal Lobe ; pathology ; Young Adult

OBJECTIVETo investigate the clinicopathologic features of focal cortical dysplasia (FCD) in patients with refractory epilepsy.

METHODSThe clinical, radiologic and pathologic features of 38 cases of FCD receiving surgical treatment in 2005 were reviewed retrospectively.

RESULTSThe mean age of disease onset was 9.2 years. The disease lasted for 11.9 years on average and often presented as complex partial seizure. Radiologic examination revealed hippocampal sclerosis, or abnormal signals in the grey matter in 21 cases. According to Palmini's classification system, the following pathologic subgroups were identified: FCD type IA (3/38), FCD type IB (20/38), FCD type IIA (5/38) and FCD type IIB (5/38). The remaining 5 cases were classified as mild cortical dysplasia. Topographically, FCD type II was often seen in the extratemporal region (8/10), predominantly in the frontal lobe (5/8). Dual pathology was identified only in cases with FCD type IB. Immunohistochemical study showed that the giant neurons, immature neurons and dysmorphic neurons were strongly positive for NeuN. A small number of balloon cells expressed nestin.

CONCLUSIONSFCD is a common cause of refractory epilepsy. FCD type IB is the predominant pathologic subtype. Associated hippocampal sclerosis is sometimes seen. Clinicopathologic differences between FCD type I and FCD type II are observed.

Adolescent ; Adult ; Antigens, Nuclear ; metabolism ; Cerebral Cortex ; pathology ; ultrastructure ; Child ; Child, Preschool ; Epilepsy ; etiology ; Female ; Humans ; Infant ; Male ; Malformations of Cortical Development ; classification ; complications ; metabolism ; pathology ; Microtubule-Associated Proteins ; metabolism ; Nerve Tissue Proteins ; metabolism ; Neurons ; metabolism ; Retrospective Studies ; Young Adult