RESUMO
Once the rabies virus has spread through the central nervous system (CNS), the virus is also transported centrifugally along axons, especially of the autonomic nervous system, to a wide range of organs including the heart. In this case report of a 49-year-old man who had been bitten by a dog in Asia, the rabies infection of cardiac nerves and cardiac muscle fibres is shown by immunohistochemistry. The neuritis cordis and rabies myocarditis can have important clinical effects on the heart rate and myocardial function and lead to blood pressure crises which are typical for the clinical course of rabies in humans.
Assuntos
Coração/inervação , Miocardite/diagnóstico , Miocardite/patologia , Neurite (Inflamação)/diagnóstico , Neurite (Inflamação)/patologia , Raiva/diagnóstico , Raiva/patologia , Animais , Mordeduras e Picadas/complicações , Encéfalo/patologia , Edema Encefálico/diagnóstico , Edema Encefálico/patologia , Diagnóstico Diferencial , Cães , Evolução Fatal , Humanos , Nervo Hipoglosso/patologia , Masculino , Pessoa de Meia-Idade , Miocárdio/patologia , Vírus da Raiva , Ribonucleoproteínas/análise , Tomografia Computadorizada por Raios X , Nervo Vago/patologiaRESUMO
Inherited cancer syndromes often involve the central and peripheral nervous system. For the surgical neuropathologist the possibility in individual patients of a familial tumour syndrome needs to be considered in the case of special tumours such as malignant peripheral nerve sheath tumour (MPNST), medulloblastoma with extensive nodularity (MBEN) or even atypical teratoid/rhabdoid tumour (AT/RT) of the brain. Furthermore, tumour location and patient age may point to a familial tumour syndrome as in the case of neurofibromatosis type 2 (NF2) with typical bilateral vestibular schwannoma in young age. This short review discusses some of the diagnostic aspects in this field relating to neurofibromatosis type 1 and 2 (NF1, NF2), as well as the two rare tumors MBEN in Gorlin-Goltz syndrome and AT/RT in particular.
Assuntos
Neoplasias/genética , Neoplasias/patologia , Neoplasias do Sistema Nervoso Central/genética , Neoplasias do Sistema Nervoso Central/patologia , Mapeamento Cromossômico , Cromossomos Humanos Par 16 , Cromossomos Humanos Par 17 , Cromossomos Humanos Par 22 , Cromossomos Humanos Par 9 , Genes da Neurofibromatose 1 , Genes da Neurofibromatose 2 , Humanos , Síndrome de Li-Fraumeni/genética , Síndrome de Li-Fraumeni/patologia , Neurofibromatose 1/genética , Neurofibromatose 1/patologia , Neurofibromatose 2/genética , Neurofibromatose 2/patologia , Neuroma Acústico/genética , Neuroma Acústico/patologia , Neoplasias do Sistema Nervoso Periférico/genética , Neoplasias do Sistema Nervoso Periférico/patologia , Tumor Rabdoide/genética , Tumor Rabdoide/patologia , Teratoma/genética , Teratoma/patologia , Esclerose Tuberosa/genética , Esclerose Tuberosa/patologiaRESUMO
The fourth edition of the WHO classification of tumours of the CNS was published in 2007. Six new entities were codified: angiocentric glioma (AG); papillary glioneuronal tumour (PGNT); rosette-forming glioneuronal tumour of the fourth ventricle (RGNT); papillary tumour of the pineal region (PTPR); spindle cell oncocytoma of the adenohypophysis (SCO); and pituicytoma. Furthermore, six histological variants of well-known brain tumours have been added, partially because they show different biological behaviour and/or prognosis: pilomyxoid astrocytoma; atypical choroid plexus papilloma; medulloblastoma with extensive nodularity; anaplastic medulloblastoma; extraventricular neurocytoma; non-specific variant of dysembryoplastic neuroepithelial tumour (DNT). The new entities and variants are discussed in this review. Moreover, the typing und grading of common-type diffuse gliomas, as well as the WHO grading system, are critically reviewed, particularly with regard to the prognostically important differential diagnosis of diffuse astrocytomas und oligodendrogliomas.
Assuntos
Neoplasias Encefálicas/patologia , Neoplasias do Sistema Nervoso Central/classificação , Neoplasias do Sistema Nervoso Central/patologia , Glioma/classificação , Glioma/patologia , Astrocitoma/classificação , Astrocitoma/patologia , Neoplasias Encefálicas/classificação , Neoplasias da Coroide/classificação , Neoplasias da Coroide/patologia , Humanos , Meduloblastoma/classificação , Meduloblastoma/patologia , Oligodendroglioma/classificação , Oligodendroglioma/patologia , Papiloma/classificação , Papiloma/patologia , Organização Mundial da SaúdeRESUMO
The prognosis of the generally benign meningiomas is mainly an issue of the likelihood of recurrence, which increases with WHO grade (7-20% in WHO grade I, 29-40% in WHO grade II, and 50-78% in WHO grade III meningiomas). Among clinical parameters the most important prognostic factor is the completeness of neurosurgical tumor resection. Among histopathological prognostic parameters the mitotic activity is the most important one. As the cutoffs of the mitotic index (MI) are defined for each grade by the WHO classification of brain tumors and because the MI can be applied as the sole grading criterion, the reliable and reproducible assessment of the MI is crucial for an appropriate risk stratification. This is provided by immunohistochemical mitosis markers, i.e., phospho-histone H3 (PHH3). The PHH3 method is superior to the conventional mitosis counting method and therefore allows a more reliable risk stratification. The Ki-67 labeling index provides additional prognostic information, especially in prognostically ambiguous meningiomas. Cytogenetically, the deletion of the short arm of one chromosome 1 (1p-) is an unfavorable prognostic parameter and is correlated with a high risk of recurrence. The enzyme reaction for alkaline phosphatase (ALPL) is a fast and efficient screening method, which strongly indicates an intact chromosome 1 in cases with a positive enzyme reaction.
Assuntos
Cromossomos Humanos Par 1 , Neoplasias Meníngeas/patologia , Meningioma/patologia , Divisão Celular , Humanos , Imuno-Histoquímica , Neoplasias Meníngeas/classificação , Neoplasias Meníngeas/epidemiologia , Neoplasias Meníngeas/genética , Meningioma/classificação , Meningioma/epidemiologia , Meningioma/genética , Mitose , Índice Mitótico , Prognóstico , Medição de Risco , Fatores de Risco , Deleção de SequênciaRESUMO
OBJECTIVE: Assessing the Ki-67 labeling index (LI) is laborious and time consuming. Therefore, an automated computer-based method was developed, which is able to identify and analyze immunolabeled and hematoxylin-stained nuclei in digital images of routine immunohistochemical slides. MATERIAL AND METHODS: The method is based on a plugin for the public domain image analysis software ImageJ, which runs on every operating system (free download at http://rsb.info.nih.gov/ij/). Percentage of Ki-67 immunostained nuclei were determined in 5 high power fields (x40) of immunostained slides (DAB detection technique, hematoxylin counterstain) of 20 Grade I, 20 Grade II, and 10 Grade III meningiomas conventionally by two independent investigators and automatically, respectively. The time effort was measured for each counting procedure. RESULTS: Enumerating conventionally or automatically did not reveal any significant differences in the mean labeling indices. Ki-67 LIs discriminated sufficiently between meningiomas of Grade I (median 1.7% Investigator 1 and 1.5% Investigator 2 vs. 1.5% automatically), Grade II (7.6%, 8% vs. 7.3%), and Grade III meningiomas (22%, 21% vs. 22%). The computer-based results correlated very closely with those obtained by manual counting (correlation coefficient = 0.98). The mean time effort for counting procedure per image was 374 s (130 s-435 s) for the conventional and 11 s (7 s-12 s) for the automated method. CONCLUSIONS: The described method can reliably assess the Ki-67 LI much faster than conventional enumerating. The computerized method has the advantages of objectivity, accuracy, repeatability, and ease of use. There is no request for special stains nor special image acquiring systems. The plugin can be downloaded at the "Morphometrie" section of http://www.uniklinikum-saarland.de/neuropathologie.
Assuntos
Biomarcadores Tumorais/análise , Núcleo Celular/patologia , Imuno-Histoquímica/métodos , Antígeno Ki-67/metabolismo , Neoplasias Meníngeas/metabolismo , Meningioma/metabolismo , Humanos , Processamento de Imagem Assistida por Computador , Neoplasias Meníngeas/patologia , Meningioma/patologia , Reprodutibilidade dos Testes , Sensibilidade e EspecificidadeRESUMO
Glioblastoma multiforme (GBM), a malignant astrocytic tumour, represents the most frequent tumour of the human brain. Nevertheless, its molecular pathology is not well understood. We utilized the immune system, which contributes to cancer protection, to help identify new GBM-related genes. By screening a human GBM cDNA library with autologous patient serum (SEREX-approach), we isolated a gene termed PHF3 (PHD finger protein 3). The gene product of PHF3 is immunogenic in GBM as tested in an allogenic patient serum screening demonstrating antibodies in 24 of 39 (61.53%) sera, whereas none of the 14 healthy persons had antibodies against PHF3. While previous SEREX studies revealed allogenic antibody responses up to 40%, our results for PHF3 represent the highest reported rate for a specific antibody response. We show that GBM patients with an antibody response against PHF3 show significant better survival than patients without PHF3-specific antibodies. Because the amino acid sequence of PHF3 contains a PHD finger (also termed LAP motif), a TFIIS homology, a proline rich region and nuclear localization signals, it supposedly functions as a transcription factor. A polyclonal antibody generated against PHF3 shows nuclear expression in most investigated formalin-fixed, paraffin embedded tissues. In GBM, PHF3 expression is concentrated in cells surrounding necroses.
Assuntos
Neoplasias Encefálicas/imunologia , Glioblastoma/imunologia , Adulto , Idoso , Sequência de Bases , Western Blotting , Neoplasias Encefálicas/sangue , Neoplasias Encefálicas/genética , Primers do DNA , Feminino , Glioblastoma/sangue , Glioblastoma/genética , Humanos , Imuno-Histoquímica , Masculino , Pessoa de Meia-Idade , PrognósticoRESUMO
The glioma amplified sequence 41 (GAS41) was previously isolated by microdissection mediated cDNA capture from the glioblastoma multiforme cell line TX3868 and shown to be frequently amplified in human gliomas. We determined the complete cDNA sequence of the GAS41 gene, demonstrated that the GAS41 protein is evolutionarily conserved, specifically at the N-terminus, and identified the yeast transcription factor tf2f domain within the GAS41 sequence. A human multiple-tissue Northern blot revealed ubiquitous expression of GAS41 with the highest expression in human brain. After generating polyclonal antibodies we found GAS41 protein expression in the nucleus of the TX3868 cell line by Western blot analysis and immunofluorescence microscopy. The nuclear localization was confirmed for several human tumors including gliomas of different grades of malignancy. In neuroblastoma however, GAS41 was found in the nucleoli but not in the nucleoplasm. Yeast two-hybrid screening of the TX3868 cell line identified the nuclear mitotic apparatus protein (NuMA), the KIAA1009 protein, and prefoldin subunit 1 (PFDN1) as potential interacting partners of GAS41. We generated a polyclonal antibody against the KIAA1009 protein and we demonstrated that the KIAA1009 protein is a nuclear protein, which appears to be co-localized with the GAS41 protein and NuMA.
Assuntos
Fatores de Transcrição/análise , Sequência de Aminoácidos , Animais , Sequência de Bases , DNA Complementar/química , Humanos , Imuno-Histoquímica , Dados de Sequência Molecular , Ligação Proteica , Fatores de Transcrição/química , Fatores de Transcrição/genéticaRESUMO
PURPOSE: Recently, we reported the isolation of six novel genes termed glioma-amplified sequences (GASs) from the glioblastoma cell line TX3868 using microdissected mediated cDNA capture (U. Fischer et al., HUM: MOL: GENET:, 5: 595-600, 1996). The aim of this study was to further characterize the gene GAS89. EXPERIMENTAL DESIGN: To determine the amplification frequency, we performed comparative PCR studies and Southern blot hybridization experiments. To identify full-length clones of GAS89 we screened a HybriZAP library. Reverse transcription-PCR was performed to isolate splice variants and to determine expression levels. RESULTS: We identified for the gene GAS89 an amplification frequency of 25% in 28 examined glioblastoma multiforme samples. Screening a HybriZAP library, we isolated an incomplete gene sequence showing identity with the gene for 25-hydroxyvitamin D(3) 1,alpha-hydroxylase. Different full-length clones were then isolated using PCR primers chosen from the 3'- and 5'-untranslated regions. As determined by sequencing, the clones represent various splice variants of the 25-hydroxyvitamin D(3) 1,alpha-hydroxylase gene. The clones encode truncated proteins but also one potentially functional enzyme variant. Reverse transcription-PCR studies revealed overexpression of several variants in glioblastoma samples with GAS89 amplification in comparison with normal brain RNA and glioblastoma without GAS89 amplification. CONCLUSIONS: This is the first report of gene amplification for 25-hydroxyvitamin D(3) 1,alpha-hydroxylase and the appearance of mRNA splice variants in glioblastoma multiforme. The endogenous expression of the 25-hydroxyvitamin D(3) 1,alpha-hydroxylase gene and the appearance of alternative splice variants reveal a new feature of the molecular pathogenesis of glioblastoma and may represent a new target for glioma therapy.
Assuntos
25-Hidroxivitamina D3 1-alfa-Hidroxilase/genética , Processamento Alternativo , Glioblastoma/genética , 25-Hidroxivitamina D3 1-alfa-Hidroxilase/biossíntese , Sequência de Bases , Encéfalo/enzimologia , Encéfalo/metabolismo , Clonagem Molecular , Amplificação de Genes , Glioblastoma/enzimologia , Glioblastoma/metabolismo , Humanos , Dados de Sequência Molecular , RNA Mensageiro/biossíntese , Homologia de Sequência do Ácido Nucleico , Células Tumorais CultivadasRESUMO
In many meningiomas, alterations of chromosome 22 can be found, and the NF2 (neurofibromatosis type 2) gene, in particular, is of great interest as a putative gene involved in meningioma. Because the NF2 gene is not mutated in all meningiomas, additional genes may be involved. Instead of looking for alterations directly at the DNA level, we used the immune response of meningioma patients to identify immunogenic antigens that may be associated with the disease. We screened a fetal brain cDNA expression library with sera pools from different patients bearing meningioma classified according to the three WHO grades, using the serological identification of antigens by recombinant expression cloning immunological screening method. Here, we report the finding of a new tankyrase-related protein. We found 16 overlapping clones with homologies to tankyrase when we screened the library with the common-type meningioma sera pool and 2 such clones when we screened the library with the atypical meningioma sera. The anaplastic meningioma sera did not identify any tankyrase-related clones. We tested some of the newly identified clones with 13 single sera, 6 of which (37.5%) reacted positively with the tankyrase-related clones. In addition, we screened the tankyrase-related clone with six sera pools from individuals without obvious disease. Although 1 of 24 (4.2%) normal sera reacted with the tankyrase-related clone, we found a striking difference in the frequency of reactivity to this clone by sera from patients bearing tumors corresponding to the three WHO meningioma grades; common-type sera was the most frequently reactive. Northern blot analysis demonstrates expression of the novel tankyrase gene in two common-type meningiomas from patients with immune response.
Assuntos
Neoplasias Meníngeas/sangue , Meningioma/sangue , Tanquirases , Sequência de Aminoácidos , Northern Blotting , Clonagem Molecular , Primers do DNA/química , Biblioteca Gênica , Humanos , Dados de Sequência Molecular , Poli(ADP-Ribose) Polimerases/genética , Reação em Cadeia da Polimerase Via Transcriptase Reversa , Homologia de Sequência de AminoácidosRESUMO
Cancer-testis (CT) genes are expressed in a variety of human cancers but not in normal tissues, except for testis tissue, and represent promising targets for immunotherapeutic and gene therapeutic approaches. Because little is known about their composite expression in human brain tumors, we investigated the expression of seven CT genes (MAGE-3, NY-ESO-1, HOM-MEL-40/SSX-2, SSX-1, SSX-4,HOM-TES-14/SCP-1, and HOM-TES-85) in 88 human brain tumor specimens. Meningiomas expressed only HOM-TES-14/SCP-1 (18% of meningiomas were HOM-TES-14/SCP-1 positive) and did not express any other CT genes. One ependymoma was negative for all CT genes tested. SSX-4 was the only CT gene expressed in oligodendrogliomas (2 of 5 cases), and it was also expressed in oligoastrocytomas (3 of 4 cases) and astrocytomas (10 of 37 cases). Astrocytomas were most frequently positive for HOM-TES-14/SCP-1 (40%) and SSX-4 (27%), followed by HOM-TES-85 (13%), SSX-2 (11%), and MAGE-3 (7%). Whereas MAGE-3 was detected only in grade IV astrocytomas, the expression of the other CT genes showed no clear correlation with histological grade. Of 39 astrocytomas, 60% expressed at least one CT gene, 21% expressed two CT genes, and 8% coexpressed three CT genes of the seven CT genes investigated. We conclude that a majority of oligoastrocytomas and astrocytomas might be amenable to specific immunotherapeutic interventions. However, the identification of additional tu-mor-specific antigens with a frequent expression in gliomas is warranted to allow for the development of widely applicable polyvalent glioma vaccines.
Assuntos
Neoplasias Encefálicas/metabolismo , Proteínas de Membrana , Neoplasias Testiculares/metabolismo , Testículo/metabolismo , Antígenos de Neoplasias/biossíntese , Astrocitoma/metabolismo , Astrocitoma/patologia , DNA Complementar/metabolismo , Ependimoma/metabolismo , Feminino , Humanos , Imuno-Histoquímica , Masculino , Meningioma/metabolismo , Proteínas de Neoplasias/biossíntese , Oligodendroglioma/metabolismo , Biossíntese de Proteínas , Proteínas Repressoras/biossíntese , Reação em Cadeia da Polimerase Via Transcriptase Reversa , Distribuição TecidualRESUMO
Meningiomas are cytogenetically characterized by loss of one chromosome 22 as a typical primary aberration and progression-associated secondary chromosome changes, of which monosomy 1p is the most common. The aim of this study was to evaluate the significance of monosomy 1p and enzyme activity loss of tissue nonspecific alkaline phosphatase (ALPL), whose gene maps to chromosome 1p36.1-p34, as parameters for the diagnosis of progression-prone meningiomas. We analyzed smear preparations of 56 meningiomas and additional paraffin sections of 17 of the cases by two-color fluorescence in situ hybridization (FISH) using the D1Z1 and D1Z2 probes and by a metaphase cytogenetic analysis of 30 of these tumors. The results were compared to clinical and morphological parameters and the expression of ALPL. Smear preparations showed deletion of 1p36 in 27% of common-type, 70% of atypical (intermediate-type), and 100% of anaplastic meningiomas. Monosomy 1p, as detected by FISH or the karyotype, was strongly associated with complete loss of ALPL activity. Intermediate-type and anaplastic meningiomas of younger patients displayed an increasing rate of cells with trisomy 1q and relative loss of 1p. The highly significant correlation of FISH results and ALPL histochemistry with clinical parameters gives evidence of their strong prognostic relevance. The complete activity loss of ALPL and the immunologically detected loss of ALPL protein in areas of meningiomas with monosomy 1p indicate a cytogenetically undetectable inactivation of the homologous Alpl allele. The apparently homozygous loss of expression of ALPL supports the notion that Alpl is a candidate tumor suppressor gene in meningiomas.
Assuntos
Fosfatase Alcalina/genética , Deleção Cromossômica , Cromossomos Humanos Par 1 , Neoplasias Meníngeas/enzimologia , Neoplasias Meníngeas/genética , Meningioma/genética , Monossomia , Biópsia , Mapeamento Cromossômico , Progressão da Doença , Feminino , Humanos , Hibridização in Situ Fluorescente , Cariotipagem , Masculino , Neoplasias Meníngeas/patologia , Meningioma/enzimologia , Meningioma/patologia , TrissomiaRESUMO
Apart from defined histomorphologic features, increased Ki-67 indices and various numeric and structural chromosome aberrations, meningiomas of the intermediate (WHO grade II, atypical meningioma) and anaplastic type (WHO grade III) are cytogenetically distinguished from common-type meningiomas (WHO grade I) by frequent loss of the distal part of the short arm of one chromosome 1 (1p-), which formerly proved to be an independent predictor of shorter recurrence-free intervals. Histochemically, loss of alkaline phosphatase activity (ALPL, liver/bone/kidney type, EC 3.1.3.1) was another frequent, specific finding in meningiomas with signs of dedifferentiation. In a prospective study including 66 meningiomas, all common-type meningiomas except one case (18/19) were reactive for ALPL, whereas 75% (30/39) of intermediate type and all anaplastic meningiomas (8/8) showed loss of enzyme activity in large areas of the tumor. Exclusively, the ALPL negative phenotype was associated with 1p loss (15/19). Our data suggest that ALPL, which is coded as a single copy gene on chromosome 1p36.1-p34, is a useful marker enzyme for the loss of a putative regulatory (tumor suppressor) gene on chromosome 1p, or that ALPL itself represents a new tumor suppressor gene homozygously inactivated in meningiomas.
Assuntos
Fosfatase Alcalina/deficiência , Cromossomos Humanos Par 1 , Deleção de Genes , Genes Supressores de Tumor , Neoplasias Meníngeas/enzimologia , Meningioma/enzimologia , Progressão da Doença , Histocitoquímica/métodos , Humanos , Imuno-Histoquímica , Estudos Prospectivos , Fatores de TempoRESUMO
The term "multiforme" in glioblastoma multiforme (GBM) indicates the highly variable histomorphology that cannot be addressed by studies on homogenized tissue probes. In order to relate genetic findings with histomorphologically distinct areas we used microdissection to procure defined cell populations from microscopic tissue sections under direct visualization. Formalin-fixed and paraffin-embedded tissue sections of 10 GBM were evaluated for intratumoral genetic heterogeneity by microdissection of multiple areas of 20-50 tumor cells and DOP-PCR of DNA isolated from the dissected cell groups, followed by comparative genomic hybridization (CGH). Microdissected cells from histomorphologically normal extratumoral blood vessels from the same slides served as controls. The individual tumors showed variable combinations of primary chromosomal gains and losses common to all studied areas of a given case along with secondary, area-specific additional aberrations. CGH displayed a wider variety of chromosomal aberrations than metaphase cytogenetics of cell cultures from the same tumors. The most frequent aberrations observed were previously unperceived gains on chromosomes 4q (8/10) and 5q (5/10). Other nonrandom aberrations were gains on 12q (6/10), 13q (6/10), and 7 (5/10), and losses of 22 (5/10). Amplifications on 7p were intratumorally heterogeneous and only found in single areas of 2 tumors. In contrast to normal extratumoral vessels, vascular proliferates in most cases demonstrated chromosomal aberrations (CGH) which were partially different from the aberrations observed in the tumor itself. The described method gives evidence of considerable intratumoral genetic heterogeneity in GBM and provides a sensitive tool for the detection of quantitative chromosomal changes that are present only regionally within a given tumor.
Assuntos
Neoplasias Encefálicas/genética , Glioblastoma/genética , Adulto , Idoso , Vasos Sanguíneos/citologia , Vasos Sanguíneos/patologia , Neoplasias Encefálicas/irrigação sanguínea , Neoplasias Encefálicas/patologia , Circulação Cerebrovascular , Aberrações Cromossômicas , Dissecação , Endotélio Vascular/patologia , Feminino , Glioblastoma/irrigação sanguínea , Glioblastoma/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Hibridização de Ácido Nucleico , Sondas de Oligonucleotídeos , Reação em Cadeia da Polimerase , Valores de ReferênciaRESUMO
A 31-year-old woman with a five year history of seizures complained of worsening fatigue and attention deficits. At age 26, neuroradiological imaging studies had shown a small, non-enhancing hypodense lesion involving the left frontobasal and subcallosal region. Follow-up CT and MRI images showed that the lesion had not changed. A stereotactic biopsy showed a dysembryoplastic neuroepithelial tumor (DNT). The lesion was subtotally resected and the diagnosis of DNT was fully confirmed. However, the distinction between oligodendroglioma, ganglioglioma, focal dysplastic cerebral cortex, and DNT was only possible with knowledge of the clinical and neuroradiological data.
Assuntos
Transtorno do Deficit de Atenção com Hiperatividade/diagnóstico , Epilepsia Tônico-Clônica/diagnóstico , Fadiga/diagnóstico , Adulto , Feminino , Humanos , Imageamento por Ressonância Magnética , Tomografia Computadorizada por Raios XRESUMO
The March COM. A 41-year-old woman presented in 1997 with diffuse abdominal pain, meteorism and intermittent diarrhea. Imaging studies revealed a focal rounded lesion in the liver. Although there was no history of progesterone or estrogen therapy, the radiographic appearance was considered to be suggestive of adenoma. The lesion was monitored by ultrasound until October 2000 when a resection was performed because of the presumed risk of a malignant transformation. H&E stained sections revealed an ectopic ependymoma that was strongly positive for GFAP. The surrounding hepatic tissue was negative for GFAP. An extensive search for a CNS manifestation or any other extraspinal localization was unrevealing. We believe we have encountered the first case of an ectopic ependymoma presenting as a solitary hepatic lesion in the absence of CNS disease. Ependymomas generally arise in the CNS in relation to the ventricular system. Extraneural metastasis from ependymomas may occasionally occur even years after detection and treatment of the primary lesion and have been the subject of several reports. In contrast, there are only anecdotal reports of primary extraneural "ectopic" ependymomas. So far those rare cases have only been found in close vicinity to the neural axis, eg, in the sacrococcygeal region, the posterior mediastinum or the ovaries and are there thought to originate from embryonic remnant cells around the neural tube. Distant metastases of ependymomas invading or arising within the extraneural lumbosacral soft tissue may occur in this situation. Here, we report what appears to be the first case of a primary ectopic ependymoma originating in the liver, with no signs of CNS or other systemic involvement.
Assuntos
Neoplasias do Sistema Nervoso Central/patologia , Ependimoma/patologia , Fígado/patologia , Dor Abdominal , Adenoma/diagnóstico , Adulto , Neoplasias do Sistema Nervoso Central/metabolismo , Ependimoma/metabolismo , Feminino , Proteína Glial Fibrilar Ácida/metabolismo , Humanos , Fígado/lesões , Imageamento por Ressonância Magnética , Tomografia Computadorizada por Raios XRESUMO
We investigated the temporal profile of the changes in regional CBF (rCBF) and intracranial pressure (ICP) during the early phase of pneumococcal meningitis in the rat. rCBF, as measured by laser-Doppler flowmetry, and ICP were continuously monitored during 6 h post infection (p.i.). Brain edema formation was assessed by brain water content determinations. Meningitis was induced by intracisternal injection of 75 microliters of 10(7) colony-forming units/ml pneumococci (n = 7). In control animals (n = 6), saline was injected. There was no change in the rCBF or ICP of controls throughout the experiment. However, there was a dramatic increase in rCBF and ICP associated with brain edema formation in untreated meningitis animals. rCBF increased to 135.3 +/- 33.8% (mean +/- SD) in the untreated animals at 1 h p.i. and reached 211.1 +/- 40.5% at 6 h p.i. (p less than 0.05 compared with controls). ICP increased from 2.9 +/- 1.4 to 10.4 +/- 4.7 mm Hg at 6 h p.i. (p less than 0.05 compared with controls). Brain water content was significantly elevated (79.69 +/- 0.24 compared with 78.94 +/- 0.16% in the control group, p less than 0.05). We investigated the effect of dexamethasone (3 mg/kg i.p.), which was given prior to the induction of meningitis (n = 3) or at 2 h after pneumococcal injection (n = 5), indomethacin (10 mg/kg i.v., n = 5), and superoxide dismutase (SOD; 132,000 U/kg i.v. per 6 h, n = 6).(ABSTRACT TRUNCATED AT 250 WORDS)
Assuntos
Circulação Cerebrovascular , Pressão Intracraniana , Meningite/fisiopatologia , Animais , Circulação Cerebrovascular/efeitos dos fármacos , Dexametasona/farmacologia , Indometacina/farmacologia , Pressão Intracraniana/efeitos dos fármacos , Masculino , Ratos , Ratos Endogâmicos , Superóxido Dismutase/farmacologiaRESUMO
OBJECTIVE: To determine systematically central nervous system and systemic complications during the acute phase of adult bacterial meningitis. DESIGN: Prospective clinical study. SETTING: University referral center, Department of Neurology. PATIENTS: A total of 86 consecutive patients between the ages of 15 and 87 years who had bacterial meningitis. MEASUREMENTS: Central nervous system complications, including brain swelling, hydrocephalus, brain abscess, subdural empyema, or subdural effusion (using computed tomography) and cerebrovascular involvement (using cerebral angiography), systemic complications, including septic shock, disseminated intravascular coagulation, adult respiratory distress syndrome, or septic or reactive arthritis, and typical complications arising during intensive care therapy. RESULTS: Of the 86 adult patients with bacterial meningitis, complications developed in 43 patients. The major central nervous system complications included angiographically documented cerebrovascular involvement (15.1% of the patients [13/86 patients]), brain swelling (14.0% [12/86]), hydrocephalus (11.6% [10/86]), and intracerebral hemorrhage (2.3% [2/86]), while septic shock (11.6% [10/86]), adult respiratory distress syndrome (3.5% [3/86]), and disseminated intravascular coagulation (8.1% [7/86]) dominated among the patients with systemic complications. Seven patients had cerebral herniation, three with a lethal course. The overall mortality was 18.6% [16/86] and was 10 of 30 (3.33%) in pneumococcal meningitis. CONCLUSIONS: Clinical and autopsy studies showed that the major determinants for the lethal outcome were primarily central nervous system complications in six patients, systemic complications in five, and a combination of both in another five. The identification of the various complications and their time of expected occurrence may help to develop additional treatment regimens in bacterial meningitis in adults.
Assuntos
Meningites Bacterianas/complicações , Infecções Pneumocócicas/complicações , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Encefalopatias/etiologia , Encefalopatias/patologia , Feminino , Humanos , Masculino , Meningites Bacterianas/patologia , Pessoa de Meia-Idade , Infecções Pneumocócicas/patologia , Estudos ProspectivosRESUMO
Cerebral hemorrhage is a rare complication of the acquired immunodeficiency syndrome. We report a case of multifocal hemorrhage in a 37-year-old homosexual man with cerebral toxoplasmosis. Histopathologic examination, performed at cerebral autopsy, confirmed the findings on CT and MRI.