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Objective@#To summarize the clinicopathological features of cystic meningioangiomatosis.@*Methods@#The clinical manifestations, imaging characteristics and pathological features of a case of cystic meningioangiomatosis were analysed, and the relevant literature was reviewed.@*Results@#A 16-year-old male patient from Xuanwu Hospital, Capital Medical University had a history of epileptic seizures for more than three months. Magnetic resonance imaging (MRI) demonstrated a cystic mass in the left frontal lobe with long T1 and long T2 signals. Extensive resection of the upper frontal gyrus was performed. The excised lesion presented with a cystic shape after incision and contained colorless translucent liquid. Microscopic examination of the lesion showed that the number of blood vessels in the local cortex of the brain tissue was increased and the vessels appeared to be branching. The blood vessel walls were surrounded by proliferative spindle cells, which were arranged in concentric circles. Immunohistochemical study revealed that those spindle cells and the cyst wall were vimentin positive. These cells had a rich reticular fibers. Ten months after the operation, the general condition of the patient was good, no epileptic seiƶure was observed, and the follow-up MRI did not reveal any residual lesion.@*Conclusions@#MRI of cystic meningioangiomatosis shows cystic space occupying. Pathological findings show typical features of meningioangiomatosis and cystic space formation. Cystic meningioangiomatosis has good prognosis after surgical resection.
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The 30th International Symposium on Amyotrophic Lateral Sclerosis-Motor Neuron Disease was held in Perth, Australia from December 4 to 6, 2019. This article mainly introduces the clinical research of this meeting, including epidemiology, non-motor symptoms, auxiliary examinations and biomarkers, etc., while the basic research includes genomics and genetics, protein metabolism abnormalities, neuroimmunity and inflammation, synapse pathology and preclinical treatment strategies,
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Objective To summarize the clinicopathological features of cystic meningioangiomatosis.Methods The clinical manifestations,imaging characteristics and pathological features of a case of cystic meningioangiomatosis were analysed,and the relevant literature was reviewed.Results A 16-year-old male patient from Xuanwu Hospital,Capital Medical University had a history of epileptic seizures for more than three months.Magnetic resonance imaging (MRI) demonstrated a cystic mass in the left frontal lobe with long T1 and long T2 signals.Extensive resection of the upper frontal gyms was performed.The excised lesion presented with a cystic shape after incision and contained colorless translucent liquid.Microscopic examination of the lesion showed that the number of blood vessels in the local cortex of the brain tissue was increased and the vessels appeared to be branching.The blood vessel walls were surrounded by proliferative spindle cells,which were arranged in concentric circles.Immunohistochemical study revealed that those spindle cells and the cyst wall were vimentin positive.These cells had a rich reticular fibers.Ten months after the operation,the general condition of the patient was good,no epileptic seizure was observed,and the follow-up MRI did not reveal any residual lesion.Conclusions MRI of cystic meningioangiomatosis shows cystic space occupying.Pathological findings show typical features of meningioangiomatosis and cystic space formation.Cystic meningioangiomatosis has good prognosis after surgical resection.
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Objective@#To investigate the clinicopathological significance of BRAF V600E and CTNNB1 gene mutations in adamantinomatous craniopharyngiomas (ACP) and papillary craniopharyngiomas (PCP).@*Methods@#The retrospective study included a total of 67 craniopharyngiomas diagnosed from October 2009 to August 2018 at Xuanwu Hospital, Capital Medical University. The immunohistochemical staining for β-catenin and BRAF V600E expression, Sanger sequencing of exon 3 of CTNNB1, BRAF mutation analysis by scorpions amplification refractory mutation system (ARMS) fluorescence quantitative PCR were performed. Univariate survival analysis was used to correlate with tumor recurrence.@*Results@#Of the 67 patients, 53 were ACPs and 14 were PCPs. Four patients underwent multiple operations and one of them presented with malignant transformation into squamous cell carcinoma. Histologically, ACPs were characterized by whorl-like cell clusters, peripheral palisaded layer, stellate reticulum, finger-shaped protrusions, ghost cells and wet keratinous substances. While PCPs usually consisted of mature squamous epithelium associated with fibrovascular stroma resulting in papillary appearance. The nuclear immunopositivity for β-catenin was observed in 73.6% (39/53) of ACPs, and it was absent in PCPs (0/14). The nuclear translocation of β-catenin usually presented at whorl-like structures or around ghost cells. Of all the cases, mutations analysis in exon 3 of β-catenin gene CTNNB1 were successful in 46 cases and 42.1% (16/38) of ACP showed CTNNB1 gene mutation, while none of the PCPs harbored CTNNB1 gene mutation (0/8). The cytoplasmic immunopositivity for BRAF V600E mutant protein was found in all PCPs (14/14) and negative in all ACPs (0/53). ARMS-PCR results showed that BRAF V600E mutations were observed in 13/14 of PCPs but not seen in ACPs (0/53). Follow-up data were available in 35 patients with duration of 2 to 120 months. Ten patients experienced recurrences after the first surgery. Upon univariate survival analysis, only subtotal excision was found to be associated with increased recurrence (P=0.032), while pathological type, postoperative radiotherapy and CTNNB1 gene mutation were not (P>0.05).@*Conclusions@#There is significant difference in the expression of BRAF V600E and CTNNB1 genes between ACP and PCP, and their immunohistochemical and molecular detection therefore can be used in the diagnosis and differential diagnoses of craniopharyngiomas.
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Objective@#To investigate the clinicopathologic features of intravascular diffuse large B-cell lymphoma (IVLBCL) in the central nervous system (CNS).@*Methods@#The clinical and radiological data of three cases of CNS-IVLBCL in Xuanwu Hospital, Capital Medical University from 2014 to 2017 were collected. And pathological and immunohistochemical features of these patients were retrospectively analyzed. The related literatures were also reviewed.@*Results@#All the three patients aged from 62 to 76 years, with duration of 4-8 months. Clinical manifestations of the three patients included dizziness, fatigue, seizures, etc. They showed intracranial multiple lesions in the frontal, temporal, parietal and corona radiata, etc. MRI demonstrated cerebral infarction or space-occupying lesions. Microscopic observation showed small vessel lumina filled with tumor cells in the white matter. Tumor cells had large, round nucleus, and prominent nucleoli. The chromatin of tumor cells was broadly granular, and the mitotic figures were visible. Tumor cells expressed B cell markers, such as CD20, CD79α, paired box protein 5 (PAX-5). CD34 staining demonstrated that tumor cells were located in the lumen of the blood vessels. Two patients died within half a year after diagnosis, and the other one was lost to follow-up.@*Conclusions@#The clinical symptoms and MRI demonstrations of CNS-IVLBCL are variable and the prognosis is extremely poor. Morphological observation and immunohistochemical phenotyping for biopsy specimens are helpful for early diagnosis and actively combining chemotherapy to prolong survival of patients.
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Objective To investigate the clinicopathologic features of intravascular diffuse large B?cell lymphoma (IVLBCL) in the central nervous system (CNS). Methods The clinical and radiological data of three cases of CNS?IVLBCL in Xuanwu Hospital, Capital Medical University from 2014 to 2017 were collected. And pathological and immunohistochemical features of these patients were retrospectively analyzed. The related literatures were also reviewed. Results All the three patients aged from 62 to 76 years, with duration of 4-8 months. Clinical manifestations of the three patients included dizziness, fatigue, seizures, etc. They showed intracranial multiple lesions in the frontal, temporal, parietal and corona radiata, etc. MRI demonstrated cerebral infarction or space?occupying lesions. Microscopic observation showed small vessel lumina filled with tumor cells in the white matter. Tumor cells had large, round nucleus, and prominent nucleoli. The chromatin of tumor cells was broadly granular, and the mitotic figures were visible. Tumor cells expressed B cell markers, such as CD20, CD79α, paired box protein 5 (PAX?5). CD34 staining demonstrated that tumor cells were located in the lumen of the blood vessels. Two patients died within half a year after diagnosis, and the other one was lost to follow?up. Conclusions The clinical symptoms and MRI demonstrations of CNS?IVLBCL are variable and the prognosis is extremely poor. Morphological observation and immunohistochemical phenotyping for biopsy specimens are helpful for early diagnosis and actively combining chemotherapy to prolong survival of patients.
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Infective endocarditis is one of the causes of cerebral infarction.Mechanical thrombectomy has been widely used in China for years.However,reports involving retrieved thrombus pathology are somewhat scarce.Here a case in which the cause of stroke was confirmed by thrombus pathology is reported.A 33-year male with a history of dilated cardiomyopathy visited the emergency because of sudden onset of left-sided weakness for 14 hours.Mechanical thrombectomy was performed,and the pathological study of the retrieved thrombus demonstrated Gram-staining positive cocci.The patient was combined with fever,septic shock,and acute kidney injury.Ultrasoundcardiography showed vegetation in the left ventricle.Therefore,he was definitely diagnosed as acute ischemic stroke due to infective vegetation embolism.
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Objective To investigate the clinical pathologic features of a distinct variant of focal cortical dysplasia (FCD) characterized by neuronal loss of layer four.Methods Between 2005 and 2017,approximately 3 000 surgeries were performed for the treatment of intractable epilepsy at Xuanwu Hospital,Capital Medical University and Yuquan Hospital,Tsinghua University.Retrospective analysis of clinic-pathological data of patients with epilepsy surgery was made and histological manifestations of neuronal loss of cortical layer four were included in this study.Results In this cohort,25 patients (22 males and three females) were identified with early onset pharmaco-resistant epilepsy and regionally circumscribed neuronal loss of cortical layer four in surgical specimens from the occipital lobe.Histologically,except for neuronal loss in cortical layer four in all cases,glial scar lesions were found in some patients.Thus the histology of those cases can be subdivided into two groups:group A (13 cases):neuronal loss of cortical layer four without glial scar lesions;and group B (12 cases):neuronal loss of cortical layer four with glial scar lesions.Due to the prominent horizontal disorganization of cortical layering and lack of any other microscopically visible principle lesion,group A should be classified hitherto as FCD International League Against Epilepsy (ILAE) type Ⅰ b,however,group B with scar lesions and cortical dysplasia around the main leision,should be classified as FCD ILAE type Ⅲd.This retrospective analysis of clinical histories revealed a perinatal distress in 20 patients (80％),suggesting an acquired pathomechanism.Magnetic resonance imaging revealed abnormal signals in the occipital lobe in all patients,and signal changes suggestive of encephalomalacia were found in 18 patients.Surgical treatment achieved favorable seizure control (Engel class Ⅰ and Ⅱ) in 18 patients (75％ among 24 available follow up).Comparion of the two groups with age at epilepsy onset (group A:5.00±2.76,group B:5.01±3.78),the proportion of perinatal distress (group A:11/13,group B:9/12) and the follow-up results (favorable seizure control of the two groups was 9/13,9/11 respectively) showed that there was no statistically significant difference between the two groups.Conculsion Neuronal loss of cortical layer four in the occipital lobe should be classified as a distinct variant of FCD ILAE type Ⅲd.
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Objective@#To investigate the prognostic impact of alterations of epidermal growth factor receptor(EGFR) and MGMT in glioblastoma.@*Methods@#The retrospective study included 161 supratentorial glioblastomas diagnosed in the Department of Pathology, Xuanwu Hospital, Capital Medical University from 2009 to 2015. EGFR and EGFRvⅢ protein expression was detected by immunohistochemistry; EGFR amplification was detected by fluorescence in situ hybridization; MGMT promoter methylation was detected by pyrosequencing. The change of molecular genetics EGFR and MGMT and outcome were assessed statistically.@*Results@#There were 161 patients, including 85 (52.8%) males and 76 (47.2%) females. The mean age was 53 years, and the median overall survival was 13 months. The integrated classification of glioblastoma included 16 IDH-mutant, 134 wild type, and 11 NOS. The rate of overexpression of EGFR protein was 32.9%(53/161), and that of EGFR amplification was 37.5%(18/48). There was high concordance between immunohistochemistry and FISH(85.4%, Kappa=0.475, P<0.01) and between the level of EGFR protein and EGFR amplification (P<0.01). Twelve cases showed EGFRvⅢ expression, and all also showed EGFR protein overexpression; 149 cases were EGFRv Ⅲ wild type, and EGFR protein overexpression was seen in 27.5%(41/149) of cases. There was no correlation between EGFR and EGFRv Ⅲ expression. Of all cases, 70.2%(106/151) showed MGMT promoter methylation by pyrosequencing. The changes of molecular genetics of EGFR and MGMT were not related. EGFR amplification and protein overexpression had no significant relationship with prognosis. Patients with EGFRv Ⅲ-mutant had shorter survival time than the EGFRv Ⅲ-wild type(P=0.014); patients with MGMT promoter methylation had better prognosis than without (PFS:P=0.002,OS:P=0.006),and MGMT promoter methylation was an independent predictor for overall survival (HR=0.269, 95%CI 0.124-0.583, P=0.001).@*Conclusions@#EGFR protein expression by immunohistochemistry correlates with the status of EGFR amplification. Patients with EGFRv Ⅲ-mutant tumors have poorer prognosis than that with EGFRv Ⅲ-wild type tumors. MGMT promoter methylation is closely associated with prognosis and an independent predictor for overall survival.
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Objective@#To investigate the clinicpathologic features and probable mechanisms of massive subcortical heterotopia.@*Methods@#Clinical data, histologic features and neuropathologic data were analyzed in five cases of massive subcortical heterotopia collected from Xuanwu Hospital, Capital Medical University from January 2014 to October 2017.@*Results@#All five patients (three males and two females) had a history of refractory epilepsy with a mean period of 15.4 years (range 7 to 21 years). The median age at surgery was 28.6 years(range 20 to 39 years). Magnetic resonance imaging showed that the lesions were located in the temporal lobe (two cases), parietal lobe (one case), both temporal and occipital lobes (one case) and both temporal and parietal lobes (one case). Pathologic examination disclosed that massive gray matter in subcortical and deep white matter with various shape and size. Moreover, one case also showed subpial and periventricular heterotopias and polymicrogyria. Polymicrogyria or hippocampal sclerosis were seen in the remaining three cases. None of the five patients experienced seizure attacks during the follow-up period.@*Conclusions@#Heterotopia is malformations due to abnormal neuronal migration. Massive subcortical heterotopia due to widespread abnormal neuronal migration is relatively rare. The mechanism of heterotopia together with polymicrogyria needs further discussion.
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Objective@#To investigate the diagnostic and prognostic implications of ATRX mutation and p53 mutation in patients with glioma.@*Methods@#The clinicopathologic and molecular features of Chinese adult glioma patients, including diffuse and anaplastic astroastrocytoma with IDH mutation, oligodendroglioma and anaplastic oligodendroglioma with IDH mutation and 1p/19q co-deletion and diffuse astroastrocytoma with IDH wild type were reviewed and tested for ATRX loss expression and p53 overexpression.@*Results@#Loss of ATRX expression was seen in 85.19% (23/27) diffuse and anaplastic astroastrocytoma with IDH mutation, higher than that of oligodendroglial tumors (0/53; P<0.01). Loss of ATRX expression was strongly linked to p53 overexpression(69.57%, 16/23). The patients who lost ATRX expression combined with normal p53 expression survived longer(P=0.013).@*Conclusions@#ATRX mutation is a molecular marker for astrocytic tumors. ATRX mutation combined with p53 mutation can predict prognosis of patients with glioma.
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Objective@#To investigate the usefulness of loss of CIC expression as the prescreening detection of 1p/19q co-deletion in the diagnosis of oligodendroglial tumors and its prognostic implication.@*Methods@#The retrospective study included 113 oligodendroglial tumors diagnosed in the Department of Pathology, Xuanwu Hospital, Capital Medical University. Expression of CIC protein was detected by immunohistochemistry, and the 1p/19q co-deletion by fluorescence in situ hybridization in all the tumors; and the correlation of the loss of protein and 1p/19q co-deletion with prognosis was assessed.@*Results@#The rate of negative CIC protein expression was 59.3% (67/113) in 113 oligodendroglial tumors. CIC protein expression was differentially lost in various gliomas, 85.7% (42/49) in pure oligodendrogliomas and 39.1% (25/64) in mixed oligodendroglial tumors (P<0.01). The loss of CIC protein expression showed a sensitivity of 76.1% (54/71), specificity 71.1% (27/38), false positive rate of 16.9% (11/65), and a false negative rate of 38.6% (17/44). In 63 cases integrated diagnosis as oligodendroglial tumors with mutant IDH and 1p/19q co-deletion, the loss of CIC protein expression was 81.0% (51/63); the sensitivity and specificity were increased to 81.0% (51/63) and 76.9% (20/26), and the false positive rate and false negative rate decreased to 10.5% (6/57) and 37.5% (12/32), respectively. By using Kaplan-Meier analysis, the CIC negative group showed a trend towards better outcome than the CIC positive group, but there was no statistical difference (overall survival: P=0.218; progression free survival: P=0.249).@*Conclusions@#Detection of the lost CIC protein expression can predict the chromosome 1p/19q co-deletion. In oligodendroglial tumors with IDH mutant and 1p/19q co-deletion, there is no relation between prognosis and CIC protein expression.
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Objective@#To study the expression and mechanism of long-chain non-coding RNA PVT1 in tumor by bioinformatics analysis and experimental verification, and to provide new ideas for the study of the pathogenesis of tumors.@*Methods@#The expression of PVT1 in 14 common tumors was downloaded from starBase v2.0 public database, which also was verified by PVT1 RNA-in situ hybridization.The upstream transcription factors, the downstream target microRNA(miRNA) for PVT1 and the target genes for the target miRNAs were predicted and analyzed by using bioinformatics based on the database of UCSC Genome Browser, HMDD v2.0, miRTar Base, JASPAR databases.@*Results@#StarBase database analysis and RNA in situ hybridization showed that PVT1 was highly expressed in kidney clear cell carcinoma and colon and rectal adenocarcinoma. PVT1 was regulated by the upstream transcription factors CREB1, Atf1, SP1, KLF5, STAT3, while it could control the expression of the downstream target miR-16. bcl-2, VEGFA, CCNE1, CCND1 and SHOC2 showed an interaction with the transcription factor of PVT1, which formed a feedback regulatory pathway.@*Conclusions@#PVT1 is highly expressed in kidney clear cell carcinoma and colon and rectal adenocarcinoma.The predictive analysis of bioinformatics demonstrates that transcription factor/PVT1/miR-16/target gene signal axon may be an important molecular mechanism, which provide a valuable clue for further functional mechanism research of long-chain non-coding RNA.
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Objective To evaluate cytological test of cerebrospinal fluid in the diagnosis of meningeal dissemination of tumor cells.Methods The clinical and imaging features of 85 cases with tumor cells diagnosed by thin-layer centrifugal cytological test of cerebrospinal fluid were retrospectively reviewed.The characteristics of cellular morphology and immunocytochemical staining were analyzed.Results The main presentations of all the patinets was meningeal irritation and neurological dysfunction.The features of the brain MRI were meningeal thicking and enhancement,intracranial abnormal signals and intracranial space occupying lesion in part of the patients.Atypical cells were found in 84 cases (98.8％) with the first sample test and immunocytochemical staining was conducted in 48 cases to identify the tissue origin.Meningeal carcinomatosis was shown to be the majority with lung cancer as the dominated tissue type and adenocarcinoma as the most common histological type.Others were lymphatic hematopoietic system (13 cases),melanomas (5 cases),primitive neuroectodermal tumor (3 cases) and glioma (1 case).In addition,12 cases were only proved to be cancer by cytological test of cerebrospinal fluid.Conclusion The thin-layer centrifugal cytological test of cerebrospinal fluid has a relatively high accuracy for detecting disseminated tumor cells of meninges and could be of great help to identify the source and type of lesion with immunocytochemical staining.
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<p><b>OBJECTIVE</b>To study the expression of autophagy-related proteins (Beclin-1, LC3 and p62) in brain tissue with malformations of cortical development and related molecular pathogenesis.</p><p><b>METHODS</b>The brain tissue of 18 cases with epileptogenic foci resection, including 6 cases of tuberous sclerosis complex (TSC), 6 cases of focal cortical dysplasia type IIb (FCD IIb) and 6 cases of focal cortical dysplasia type I (FCD I), were retrieved. Immunohistochemical study for Beclin-1, LC3 and p62 proteins was performed. The degree of positivity for Beclin-1 and LC3 proteins was compared. Western blot was used to quantitatively analyze the LC3 protein in focal lesion of each disease groups.</p><p><b>RESULTS</b>Immunohistochemical study showed that the three proteins were mainly expressed in the dysmorphic neurons and balloon cells/giant cells of TSC and FCD IIb. The positivity was more intense in the dysmorphic neurons than the other cell types. Immunostaining for Beclin-1 showed granular or diffuse cytoplasmic positivity, in addition to the strong expression in axons. On the other hand, LC3 showed diffuse or perinuclear cytoplasmic expression. The staining for p62 was mainly cytoplasmic or perinuclear and sometimes nuclear. In FCD type I, only individual cells showed positive expression for the three proteins. The number of Beclin-1 and LC3-positive cells was larger in TSC group, followed by FCD IIb group and FCD I group.And there were significant differences between TSC group and FCD I group, as well as FCD IIb group and FCD I group (P<0.05). Quantitative expression of LC3 protein by Western blot showed smaller amount in TSC group, followed by FCD IIb group and FCD I group.</p><p><b>CONCLUSIONS</b>The dysmorphic neurons and balloon cells/giant cells of TSC and FCD IIb show abnormality in autophagy, resulting in intracytoplasmic protein accumulation. There are differences in molecular pathogenesis in these cell types.</p>
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Objective To explore the clinicopathological features and imaging characteristics of non-Langerhans cell histiocytosis in central nerve system,thus to facilitate the diagnosis and differential diagnosis.Methods A total of ten cases were enrolled in the study,with seven cases of Rosai-Dorfman disease(RDD) and three cases of xanthoma disseminatum (XD).Data on the clinicopathological features,imaging,immunophenotype and prognosis were collected and analyzed.Results Seven patients with RDD,5 males and 2 females with the mean age of 46.7 years old,all presented as dural-based or intraparenchymal hypo-to isointense lesions on T1 and T2 with post-contrast enhancement.The polymorphous admixture of histiocytes,lymphocytes and plasma cells was observed in a fibrous stroma,with emperipolesis of some histiocytes.The immunohistostaining of CD11c,CD68,MAC387 and S-100 was positive in the histiocytes,while the staining of CD1α was negative.Five patients recovered after the operation,while one patient died of the disease.All the 3 XD patients were female,with the median age of 20.7 years old.All XD patients presented as multiple intraparenchymal hypointense lesions on T1 and hyperintense lesions on T2 with post-contrast enhancement.The infiltration of foam-like histiocytes,a few Touton giant cells,lymphocytes and eosnophils was observed in all XD patients.The immunohistostaining of CD68 and CD11c was positive in the histiocytes and that of MAC387 partly positive,while the staining of S-100 and CD1α was negative.One XD patient survived well,while another one died of the disease.Conclusions The diagnosis of RDD and XD should be based on their typical morphology and immunophenotype and should be differentiated from Langerhans cell histiocytosis and other non-Langerhans cell histiocytosis.Non-Langerhans cell histiocytosis in central nerve system often presents untypical clinical presentation and imaging features,thus the communication and cooperation between clinician and pathologist is needed.
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<p><b>OBJECTIVE</b>To study the expression and prognostic significance of ERG and SPINK1 expression in endocrine-treated prostatic cancer.</p><p><b>METHODS</b>Prostatic needle biopsies from 118 prostatic cancer patients primarily treated with endocrine therapy were reviewed. Immunohistochemical study for ERG and SPINK1 protein was carried out.</p><p><b>RESULTS</b>Co-expression of ERG and SPINK1 was not observed. The frequency of ERG protein expression in the 118 biopsies studied was 9.3% (11/118). The positive expression correlated with T stage (P=0.04) but not with patient age at diagnosis, prostatic specific antigen level, Gleason's score, M stage, tumor area and progression-free survival. Positive expression of SPINK1 was demonstrated in 11.0% (13/118) of the biopsies. SPINK1-positive cases carried a significantly shorter progression-free survival, as compared with SPINK1-negative cases (P=0.022). The expression was not associated with any other clinicopathologic variables. The following expression pattern showed statistically significant correlation with the clinical progress (P=0.029): ERG+/SPINK1- (11/118, 9.3%), ERG-/SPINK1+ (13/118, 11.0%) and ERG-/SPINK1- (94/118, 79.7%).</p><p><b>CONCLUSIONS</b>ERG and SPINK1 proteins are mutually exclusive.SPINK1 expression is associated with more aggressive clinical behavior and can serve as a prognostic biomarker in prostatic cancer.</p>
Asunto(s)
Anciano , Anciano de 80 o más Años , Humanos , Masculino , Persona de Mediana Edad , Antineoplásicos Hormonales , Usos Terapéuticos , Proteínas Portadoras , Metabolismo , Supervivencia sin Enfermedad , Estudios de Seguimiento , Inmunohistoquímica , Clasificación del Tumor , Estadificación de Neoplasias , Pronóstico , Neoplasias de la Próstata , Quimioterapia , Metabolismo , Patología , Transactivadores , Metabolismo , Regulador Transcripcional ERG , Inhibidor de Tripsina Pancreática de KazalRESUMEN
Objective To recognize the developments and achievements of the clinical neuropathology in China from 1955 to 2009.Methods Retrospective survey of literatures related clinical neuropathology published in Chinese Journal of Neurology (CJN) from the first issue in 1955 to the No.12 issue in 2009 was conducted; Total literatures of CJN and literatures of the clinical neuropathology were respectively counted and the percentage of the latter was analyzed in different periods; Proportion of autopsy and biopsy literatures was also analyzed.According to categories of nervous system diseases,their relative percentages were respectively counted.The percentages of clinical neuropathological literatures and autopsy literatures of CJN were compared with the data of the same period of J Neurol Neurosurg Psychiatry (JNNP).Results (1) The percentages of clinical neuropathological literatures in total literatures of CJN were 18.11％ (65/359),20.81％ (103/495),17.05％ (22/129),15.50％ (230/1484),11.58％ (176/1520) and 16.04％ (309/1927),respectively,in 1955-1959,1960-1969,1970-1979,1980-1989,1990-1999,2000-2009.The percentage variation trend in different periods from 1955 to 2009 had significant difference (trend x2 =7.977,P =0.005) ; But there was no significant difference in the variation trend of the percentage of clinical neuropathological literatures between CJN and JNNP from 1955 to 2009.(2) The percentages of autopsy in the total clinical neuropathological literatures of CJN from 1995 to 2009 appeared gradually to decline; Compared with JNNP,the percentages of autopsy in the total clinical neuropathological literatures of CJN during 1980-2009 were lower,and there was significant difference in the variation trend of the percentage of autopsy in the total clinical neuropathological literatures between CJN and JNNP.(3) The main types of neurological diseases in the neuropathological literatures were tumors,infectious diseases and cerebrovascular diseases from 1955 to 1989,while myopathies became the most main disease type during 1990-2009; Compared with the same period JNNP,the clinical neuropathological literatures of the rare and difficult diseases and neurodegenerative diseases in CJN were relatively less.Conclusions Neuropathology is important in the clinical neuroscience research.From 1955 to 2009,main achievements of clinical neuropathological researches in China mainland lie in tumors,cerebrovascular diseases,infectious diseases and myopathies.But in recent years,due to the lack of autopsy cases,it is to the disadvantage of exploring pathogenesis of neurodegenerative diseases and understanding some rare and difficult diseases.
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Objective To study a histopathological classification system for hippocampal cell loss in patients suffering from mesial temporal lobe epilepsy.Methods Fifty-one surgically resected specimens were microscopically examined with respect to neuronal cell loss in hippocampal subfields CA1—CA4.Clinical data including age at first seizure,with or without initial precipitating injuries (IPIs),latecy,epilepsy duration,Engel score of half and one year after surgery were collected to analyse the clinical characteristics between different pathological types.Eight additional hippocampal specimens obtained from neurologically healthy autopsies served as controls.Results (1)Five distinct patterns were recognized within a consecutive cohort of anatomically well-preserved surgical specimens.Type 1 a (21/51,41.18 ％):severe cell loss in CA1 and moderate neuronal loss in all other subfields excluding CA2; Type 1b(18/51,35.2％):severe cell loss in all sectors; Type 2(9/51,17.65％):severe neuronal loss restricted to sector CA1 ;Type 3 (1/51,1.96％):severe neuronal loss restricted to the hilar region; no mesial temporal sclerosis (2/51,3.92％):a group comprised hippocampi with neuronal cell densities not significantly different from age matched autopsy controls.(2)The patients of type 1 a and 1 b had younger age of first onset,longer latency and duration,more frequency of IPIs,the proportion of ourrence of febrile seizures in type 1a was 10/19,type 1b was 7/16,type 2 was 4/7,type 3 was 0 (x2 =11.790,P =0.019).(3) The patients of type 1a and 1 b had better postsurgical outcome.Conclusion Type 1 is the most common type of mesial temporal sclerosis,which have better postsurgical outcome than the other 3 types.
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Objective To compare the expression and distribution of drug resistance proteins Pglycoprotein (P-gp) and lung resistance protein (LRP) in brain tissues of patients with refractory epilepsy and to investigate the relationship between expression of drug resistance proteins and concentration of antiepileptic drug carbamazepine (CBZ).Methods We included the brain tissues of 26 cases with refractory epilepsy who had in the experiment.They had following pathologic diagnosis: focal cortical dysplasia (FCD) Ⅰb (n =10),FCD Ⅱ a (n =4),FCD Ⅱb (n =2) and brain tumorsincluding ganglioglioma (n =6) and dysembryoplastic neuroepithelial tumor(n =4).Immunohistochemistry staining using EnVision system was used to reveal the expression location of P-gp and LRP,and Western blot in SDS-polyacrylamide gel was used to quantitatively analyze the expression of P-gp and LRP.Fluorescence polarization immunoassay was used to determine concentration of CBZ.Results Both P-gp and LRP performed(μg/ml) prominent overexpression in brain tissues of patients with refractory epilepsy,especially in the lesions of both FCD type Ⅱ (P-gp: 2.593 ±0.829 vs 1.711 ±0.292,t =-2.201,P=0.028;LRP:1.352 ±0.445 vs 1.179 ±0.593, t =-2.516,P =0.028, respectively)and tumor(P-gp:1.567 ±0.092 vs 0.775 ± 0.101, t =2.698, P =0.024; LRP: 1.091 ± 0.239 vs 0.825 ± 0.297, t =3.997, P =0.003respectively).The concentration of CBZ in lesions of brain tumors were lower than which in surrounding regions(0.848 ±0.726 vs 0.948 ±0.785, t =-3.056,P =0.014), while P-gp and LRP were higher than which in surrounding regions in 80％ cases.There was no relationship between expression of drug resistance proteins and concentration of CBZ.Conclusions In processing of drug resistance, P-gp and LRP play important roles.However, there is no correlation between expression of drug resistance proteins and concentration of CBZ, suggesting epilepsy drug resistance to be a complicated mechanism.