Glioblastoma de células gigantes: informe de un caso / Giant cell glioblastoma: case report

Antecedentes: El Glioblastoma (GB) o astrocitoma grado IV, es un tumor agresivo que se origina de células gliales, con alto grado de malignidad, prevalencia menor al 1% en fosa posterior e incidencia menor al 0.5% de todos los GB. Actualmente se describen alrededor de 75 casos a nivel mundial. Descripción del caso clínico: Femenina, 24 años, referida a emergencia de Neurocirugía del Hospital Escuela Universitario, presentó cefalea holocraneana intensa, vómitos, náuseas, visión borrosa, vértigo y anorexia. Al examen neurológico mostró discreta adiadococinesia derecha y signos de papiledema. La tomografía axial computarizada cerebral evidenció lesión heterogénea en vermis extendido a hemisferio cerebeloso derecho, por lo que se realizó craniectomía suboccipital, abordaje transcerebelar, con citorreducción tumoral, encontrando masa vascularizada con componente quístico. Estudio anatomopatológico evidenció glioblastoma multiforme variante de células gigantes, confirmado con tinción de inmunohistoquímica (PFGA, CD34+ y vimentina). Paciente con buena evolución clínica postquirúrgica, egresada sin déficit neurológico. 16 meses después, presentó síndrome de recidiva tumoral y complicaciones, por lo que se reintervino en 4 ocasiones, posterior a recibir 30 dosis de radioterapia y 12 ciclos de quimioterapia, se reingresó con deterioro neurológico progresivo, signos meníngeos y síndrome de Parinaud, escala de Karnofsky (30 puntos), realizándose derivación ventrículo-peritoneal por compresión del IV ventrículo e hidrocefalia obstructiva secundaria, luego desarrolló neumonía intrahospitalaria, falleciendo a las dos semanas. Conclusiones: Es importante identificar la variante biológica del glioblastoma de forma temprana, para determinar pronóstico y acciones terapéuticas que influirán en la calidad de vida, así como la supervivencia...(AU)

Homeobox B2 is a potential prognostic biomarker of glioblastoma

SUMMARY OBJECTIVES HOXB2 is a new prognostic indicator for lung cancer. But it is unclear whether HOXB2 holds an effect in glioblastoma (GBM) progression. The purpose of this article was to probe the influences of HOXB2 on GBM pathogenesis. METHODS HOXB2 expression level and prognostic power in GBM patients were analyzed. Then the mRNA and protein expression levels of HOXB2 in GBM cell lines were tested by qRT-PCR and western blotting. Cell proliferation, invasion, and migration were determined by CCK8 and transwell assay, severally. The protein levels of PI3K/AKT-pathway associated proteins were analyzed by western blotting. RESULTS The results indicated that HOXB2 was distinctly overexpressed in GBM patients and high expression of HOXB2 was related to a poor prognosis. Moreover, the expression of HOXB2 was higher in all GBM cell lines U251, U-87MG, GOS-3 than that in HEB cells (normal control). Meanwhile, decreased expression of p-PI3K and p-AKT were identified after HOXB2 knockdown. CONCLUSIONS These data demonstrated that HOXB2 had a vital role in GBM progression and could serve as a promising target for GBM treatment.

RESUMO OBJETIVOS A HOXB2 é um novo indicador prognóstico para o câncer de pulmão. Mas não está claro se a HOXB2 tem algum efeito na progressão do glioblastoma (GBM). O objetivo deste artigo foi sondar as influências da HOXB2 na patogênese do GBM. MÉTODOS Foram analisados o nível de expressão e o poder prognóstico da HOXB2 em pacientes com GBM. Em seguida, os níveis de expressão proteica e mRNA da HOXB2 em linhagens de células de GBM foram testados por qRT-PCR e western blotting. A proliferação, a invasão e migração celular foram determinadas por CCK8 e ensaios transwell, várias vezes. Os níveis proteicos das proteínas associadas à via PI3K/AKT foram analisados pelo método western blotting. RESULTADOS Os resultados indicaram que havia uma clara superrexpressão da HOXB2 em pacientes com GBM e que a alta expressão da HOXB2 estava relacionada a um prognóstico negativo. Além disso, a expressão da HOXB2 foi mais elevada em todas as linhagens de células do GBM U251, U-87MG, GOS-3 do que nas células HEB (controle normal). Entretanto, a diminuição da expressão de P-PI3K e p-AKT foi identificada após a redução da expressão da HOXB2. CONCLUSÕES Esses dados demonstram que a HOXB2 desempenha um papel vital na progressão do GBM, podendo ser um alvo promissor para o tratamento do GBM.

Síntese, avaliação biológica e modelagem molecular de potenciais antitumorais análogos da sanguinarina planejados por simplificação molecular / Synthesis, biological evaluation and molecular modeling of potential antitumors analogues from sanguinarine designed by molecular simplification

A sanguinarina é um alcaloide capaz de inibir Bcl-xL, uma proteína antiapoptótica que se encontra superexpressa em linhagens tumorais e que está frequentemente relacionada à resistência destas frente a quimioterápicos antineoplásicos. No intuito de identificar potenciais agentes antitumorais, o objetivo deste trabalho foi sintetizar três séries de análogos da sanguinarina planejados por simplificação molecular e avaliar sua atividade biológica. Dez N-benzil-naftil-aminas (3a-e; 4a-e) e dez arilisoquinolinas (6a-e; 7a-e) foram sintetizadas em duas a três etapas reacionais, utilizando-se métodos de aminação redutiva e acoplamento de Suzuki. Insucesso na etapa de reação de Heck impossibilitou a síntese da terceira série, benzofenantridínica, apesar de testadas diversas condições reacionais. Avaliação da citotoxicidade em linhagens de glioblastoma U87MG revelou que a série N-benzilnaftil-amina apresenta melhor atividade quando comparada às aril-isoquinolinas, sendo para ambas, observada atividade superior à temozolamida, principal fármaco para o tratamento de glioblastoma. Estudos em linhagem não tumorigênica MRC-5 demonstraram que os análogos foram significativamente superiores à sanguinarina em relação à seletividade. Os compostos mais mais promissores, 4a e 6e, induziram morte celular por apoptose e causaram despolarização da membrana mitocondrial, indicando morte apoptótica pela via extrínseca. Ademais, 4a interrompeu o ciclo interrompeu o ciclo celular na fase G2/M, indicando que o mesmo seria um agente ciclo celular específico. Simulações de dinâmica molecular sugerem que os compostos interagem com a proteína Bcl-xL principalmente por interações hidrofóbicas, e que o composto 4a apresentaria afinidade com o alvo semelhante à sanguinarina, embora esta tenha apresentado atividade superior em células U87. Perspectivas incluem estudos das vias de indução de morte celular, além da expansão do painel de células. Conclui-se, portanto, que os análogos da sanguinarina representam um arcabouço a ser explorado pelos químicos medicinais no desenvolvimento de potenciais antineoplásico

Sanguinarine is an alkaloid able to inhibit Bcl-xL, an antiapoptotic protein which is overexpressed in tumor cells and related to their resistance against antineoplastic chemotherapy. Regarding to develop potential antitumor agents, the aim of this work was the synthesis of three series of sanguinarine analogues designed by molecular simplification and their biological evaluation. Ten N-benzyl-naphtyl-amines (3a-e; 4ae) and ten aryl-isoquinolines (6a-e; 7a-e) were synthesized in two or three reaction steps through reductive amination and Suzuki coupling. Failure about Heck-type reaction had impaired the synthesis of the thirth series, benzophenanthridine, although several conditions were tested. Cytotoxicity evaluation against U87MG glioblastoma cell line showed that N-benzyl-naphtyl-amines are more active than aryl-isoquinolines and both series were superior to temozolamide, the main drug for glioblastoma treatment. Tests against non-tumorigenic cell MRC-5 indicated that the analogues were significantly superior to sanguinarine regarding selectivity. The most promising compounds, 4a e 6e, induced cell death by apoptosis and mitochondrial membrane depolarization, indicating apoptotic death by extrinsic pathway. 4a provide cell cycle arrest at G2/M phase, suggesting that it is a specific cell cycle agent. Molecular dynamics suggested that compounds interact with Bcl-xL mainly by hydrophobic interactions and 4a has affinity to the protein like sanguinarine, although the last showed superior activity against U87 cells. Perspectives include mechanistics studies about cell death pathway and expanding cell panel. In conclusion, sanguinarine anlogues represent a scaffold to be explored by medicinal chemists to the development of potential antitumor agent

Immunohistochemical Analysis of ATRX, IDH1 and p53 in Glioblastoma and Their Correlations with Patient Survival

Glioblastoma (GBM) can be classified into molecular subgroups, on the basis of biomarker expression. Here, we classified our cohort of 163 adult GBMs into molecular subgroups according to the expression of proteins encoded by genes of alpha thalassemia/mental retardation syndrome X-linked (ATRX), isocitrate dehydrogenase (IDH) and TP53. We focused on the survival rate of molecular subgroups, depending on each and various combination of these biomarkers. ATRX, IDH1 and p53 protein expression were evaluated immunohistochemically and Kaplan-Meier analysis were carried out in each group. A total of 15.3% of enrolled GBMs demonstrated loss of ATRX expression (ATRX-), 10.4% expressed an aberrant IDH1 R132H protein (IDH1+), and 48.4% exhibited p53 overexpression (p53+). Survival differences were statistically significant when single protein expression or different combinations of expression of these proteins were analyzed. In conclusion, in the case of single protein expression, the patients with each IDH1+, or ATRX-, or p53- GBMs showed better survival than patients with counterparts protein expressed GBMs. In the case of double protein pairs, the patients with ATRX-/p53-, ATRX-/IDH1+, and IDH1+/p53- GBMs revealed better survival than the patients with GBMs with the remained pairs. In the case of triple protein combinations, the patients with ATRX-/p53-/IDH+ showed statistically significant survival gain than the patients with remained combination of proteins-expression status. Therefore, these three biomarkers, individually and as a combination, can stratify GBMs into prognostically relevant subgroups and have strong prognostic values in adult GBMs.

Validation of the Effectiveness and Safety of Temozolomide during and after Radiotherapy for Newly Diagnosed Glioblastomas: 10-year Experience of a Single Institution

This study was performed to validate the effectiveness and safety of concurrent chemoradiotherapy and adjuvant therapy with temozolomide for newly diagnosed glioblastoma multiforme as a standard treatment protocol. Between 2004 and 2011, patients newly diagnosed with glioblastoma who were treated with temozolomide during concurrent chemoradiotherapy and adjuvant chemotherapy were included from a single institution and analyzed retrospectively. The primary endpoint was overall survival, and the secondary endpoints were progression-free survival, response, and safety. A total of 71 patients were enrolled in this study. The response rate was 41% (29/71), and the tumor control rate was 80% (57/71). In the 67 patients who completed the concurrent chemoradiotherapy with temozolomide, the median overall survival was 19 months and the 1- and 2-yr overall survival rates were 78.3% and 41.7%, respectively. The median progression free survival was 9 months, and the 1- and 2-yr progression free survival rates were 33.8% and 14.3%, respectively. The mean duration of survival after progression of disease in salvage treatment group was 11.9 (1.3-53.2) months. Concurrent chemoradiotherapy with temozolomide resulted in grade 3 or 4 hematologic toxic effects in 2.8% of the patients. The current protocol of temozolomide during and after radiation therapy is both effective and safe and is still appropriate as the standard protocol for treatment of glioblastoma. An active salvage treatment might be required for a better prognosis.

Metaplasia óssea no gliossarcoma: um achado histológico pouco usual. Relato de caso / Osseous metaplasia in gliosarcoma: an unusual histologic finding. Case report

Gliosarcoma (GS) is a malignant neoplasm of the central nervous system that has coexisting glial and mesenchymal components. GSs are rarely related to osseous metaplasia. The authors report a case of GS in a male patient presenting apathy and catatonia. Computed tomography/magnetic resonance imaging showed an expansive process affecting the left frontal lobe. At microscopy, a malignant glioma constituted by highly atypical glial cells intermingled with spindle-shaped cells was identified. The lesion showed areas of necrosis with pseudopalisading formation, focus of osseous metaplasia, and positive immunoexpression of S100, CD99 and vimentin in both elements. Only the sarcomatous component exhibited negative immunoexpression of glial fibrillary acidic protein (GFAP). The diagnosis of GS was then established...

Gliossarcoma (GS) é uma neoplasia maligna do sistema nervoso central que apresenta coexistência de componentes glial e mesenquimal. Raramente, os GS estão associados à metaplasia óssea. Os autores descrevem um caso de GS em paciente masculino apresentando apatia e catatonia. A tomografia computadorizada e a ressonância magnética mostraram um processo expansivo comprometendo o lobo frontal esquerdo. À microscopia, foi identificado um glioma maligno constituído por células gliais extremamente atípicas entremeadas com células fusiformes. A lesão mostrava áreas de necrose com formação de pseudopaliçada, focos de metaplasia óssea e expressão imuno-histoquímica positiva para S100, CD99 e vimentina em ambos os componentes. Somente o componente sarcomatoso exibiu imunoexpressão negativa para proteína glial fibrilar ácida (GFAP). O diagnóstico de GS foi, então, estabelecido...

Glioblastoma multicêntrico originado em dois locais incomuns: cerebelo e tálamo / Multicentric glioblastoma arising in two unusual sites: cerebellum and thalamus

Multicentric glioblastomas (MGBM) arising in infra/supratentorial regions are uncommon lesions. The authors report a case of MGBM in a 61 year-old female patient, who presented a sudden onset of left hemiplegia. The magnetic resonance imaging (MRI) showed two expansive large lesions affecting cerebellum and thalamus, with strong contrast enhancement. The patient underwent resection of the cerebellar lesion. Microscopy revealed a high grade glial neoplasm exhibiting high mitotic index, areas of necrosis and microvascular proliferation. The neoplastic cells showed positive immunoexpression for glial fibrillary acidic protein (GFAP). The morphological findings were consistent with glioblastoma (GBM). The patient was referred to radiotherapy, with discrete signs of tumor regression after a 60-day clinical follow-up.

Glioblastomas multicêntricos (GBMM) originados em regiões infra/supratentoriais são lesões incomuns. Os autores relatam um caso de GBMM em paciente do sexo feminino, 61 anos de idade, que apresenta quadro súbito de hemiplegia esquerda. O exame de ressonância magnética (RM) mostrou duas lesões expansivas volumosas, com forte impregnação pelo contraste no cerebelo e no tálamo. A paciente foi submetida à ressecção da lesão cerebelar. À microscopia, foi identificada uma neoplasia glial de alto grau exibindo alto índice mitótico, áreas de necrose e proliferação microvascular. As células neoplásicas revelaram imunoexpressão positiva para proteína glial acídica (GFAP). O conjunto das alterações morfológicas foi consistente com glioblastoma. A paciente foi encaminhada para radioterapia, com sinais discretos de regressão tumoral após acompanhamento clínico de 60 dias.

Spinal Cord Glioblastoma Induced by Radiation Therapy of Nasopharyngeal Rhabdomyosarcoma with MRI Findings: Case Report

Radiation-induced spinal cord gliomas are extremely rare. Since the first case was reported in 1980, only six additional cases have been reported.; The radiation-induced gliomas were related to the treatment of Hodgkin's lymphoma, thyroid cancer, and medullomyoblastoma, and to multiple chest fluoroscopic examinations in pulmonary tuberculosis patient. We report a case of radiation-induced spinal cord glioblastoma developed in a 17-year-old girl after a 13-year latency period following radiotherapy for nasopharyngeal rhabdomyosarcoma. MRI findings of our case are described.

Report of two brain cancer cases with survey more than 15 years

Brain tumors are considered in regarded to their size, position and aggressive manner. Brain tumors cause several neurologic symptoms. For instance, they increase intracranial pressure due to local damage and displacement of surrounding tissues. In this paper, two glioblastoma multiform cases with more than 15 year- survival have been reported. The first patient was a 52 year- old woman who suffered from seizure. The CT scan demonstrated a focal low density in the left temporoparietal lobe, and Carbamazepine was prescribed. However, after 12 years, the patient started to suffer from seizures with transient awareness repletion and vomiting. Therefore, daily injection of 16 mg of dexamethason in two divided dosages was prescribed. Fourteen years after illness, biopsy with stereotaxy method was used and glioblastoma multiform was diagnosed by a pathologist. Unfortunately, this patient died due to progression of her illness 16 years after beginning of the seizures. The second patient was a 47 year- old man who also suffered from seizure. In the CT scan, a low density lesion was observed in his left parietal lobe. The patient was treated with 400 mg of Carbamazepine per day in two divided dosages. Dosage of drug was increased to 1200 mg per day. After one year, due to drug resistance, Phenytoin and Phenobarbital were also added to Carbamazepine because of repeated seizures. After 15 years, the soberness disorder of the patient increased and CT scan was repeated. However, due to increase in the tumor size, hydrocephaly surgery was performed and the pathologist reported glioblastoma multiform. Following the surgery, radiotherapy and chemotherapy were also used. This patient is in a good condition now and he has no serious problems. Glioblastoma multiform is a high degree astrocytic tumor. In this paper, two patients who were afflicted with glioblastoma multiform in fourth and fifth decades of their life were presented. The life span of these patients is considerable in contrast to several articles which indicated that a- five -year life span is rare in patients with glioblastoma multiform

Desarrollo de glioblastoma multiforme tras una hemorragia intracerebral: reporte de un caso / Development of glioblastoma multiforme after intracerebral hemorrhage: case report

Varios factores han sido implicados como posibles causantes del desarrollo de tumores cerebrales; son pocos los autores que han proporcionado evidencia de la etiología traumática de un tumor cerebral. Presentamos el caso de un paciente masculino de 30 años quien se presento con un cuadro clínico de cefalea intensa de 15 días de evolución. Los estudios imagenológicos (TAC, RMN) revelaron lesión frontal intra axial. Se le realizo resección total de la lesión, cuyo resultado de patología fue compatible con glioblastoma multiforme. El paciente tenía antecedente de hemorragia intracerebral espontanea hacia 7 meses en el mismo lugar del tumor. Basados en la literatura en relación a glioblastoma multiforme y lesión traumática o vascular cerebral, hacemos una revisión crítica de ella.

Prognostic factors in glioblastoma multiforme / Fatores prognósticos no glioblastoma multiforme

Objective: To study if the prognosis variables such as age, the Karnofsky Performance Status (KPS), extension of tumor removal by surgery, radiotherapy and tumor volume influenced the survival of patients with glioblastoma multiforme (GBM). Method: Retrospective analysis of GBM patients operated at Hospital dos Servidores do Estado between 1998 and 2008. Results: We could observe that age, the KPS and radiotherapy influenced the survival. The other variables did not have any prognosis implications. Conclusions: Despite many researches and many improvements regarding the diagnosis and the surgical techniques, the survival of patients with GBM has not changed in the last 30 years and is a therapeutic challenge. The surgical resection followed by radiotherapy is the standard treatment for patients with GBM. The importance of each variable in the patient's prognosis is still to be established in the multivariate analyzes.

Objetivo: Observar se os fatores prognósticos dos pacientes com glioblastoma multiforme (GBM), como a idade, o Karnofsky Performance Status (KPS), a extensão da remoção tumoral, a realização de radioterapia e o volume tumoral, influenciam a sobrevida dos pacientes. Método: Realizamos uma revisão retrospectiva dos prontuários dos pacientes operados no Hospital dos Servidores do Estado do Rio de Janeiro no período de 1998 a 2008. Resultados: Observamos que a idade, a realização da radioterapia e o KPS influenciaram a sobrevida dos pacientes. As demais variáveis não tiveram implicações prognósticas. Conclusões: Apesar de inúmeras pesquisas, a sobrevida dos pacientes com GBM praticamente não se alterou nos últimos 30 anos, permanecendo como um desafio terapêutico. A ressecção cirúrgica complementada com radioterapia ainda é o tratamento de escolha. O papel de cada variável no prognóstico dos pacientes ainda está por ser definida nas análises multivariadas.

Glioma multifocal multicéntrico / Multifocal multicentric glioma

El glioblastoma multiforme es el más habitual y agresivo de los tumores gliales del sistema nervioso central, sin embargo, infrecuentemente se expresa con lesiones múltiples que pueden definirse como multifocales o multicéntricas, en relación a su origen y capacidad de propagación. Esta presentación intenta analizar las peculiaridades clínicas y los hallazgos imagenológicos de un paciente portador de un glioblastoma multiforme con lesiones supra e infratententoriales, de aparición sincrónica y metacrónica. Llamó la atención en él, que importantes masas tumorales de la protuberancia y mesencéfalo no comprometieran los pares craneales. También fue significativa la visualización en el cuerpo calloso de la propagación selectiva del glioma a través de fibras de conexión. Se concluye que las manifestaciones tanto clínicas como imagenológicas de este paciente son excepcionales por la ausencia de signología esperable por la localización de las múltiples lesiones infra y supratentoriales del tumor, y la visualización del modo de propagación.

Glioblastoma multiforme is the most common and most aggressive glial tumor of central nervous system, however it is infrequently expressed with multiple lesions that can be defined as multifocal or multicentric, relative to their origin, and spread capacity. This presentation makes an effort to analyze clinical peculiarities and radiographic findings of a patient suffering from a glioblastoma multiforme with supra and infratentorial lesions, that appeared synchronic and metachronically. In this case, drew our attention that important tumor masses at pons and midbrain did not compromise cranial nerves. Also, it was significant the visualization at corpus callosum of glioma's selective spreading through connection fibers. It is concluded that both clinical and radiographic manifestations from this patient are exceptional, because of the absence of expected signology from the multiple infra and supratentorial lesions, and the visualization of the way of spreading.

Glioblastoma: experiência uni-institucional com 48 pacientes tratados com cirurgia, radioterapia e quimioterapia / Glioblastoma: single institutional experience with 48 patients treated with surgery, radiotherapy and chemotherapy

OBJETIVO: Identificar fatores prognósticos e avaliar a evolução clínica de pacientes com diagnósticode glioblastoma submetidos a cirurgia e radioterapia, com ou sem quimioterapia adjuvante. MATERIAL E MÉTODO: trabalho retrospectivo com 48 pacientes portadores de glioblastoma tratadosno período de 1997 a 2007. Todos os pacientes foram classificados segundo critérios do recursive partitioning analysis (RPA). RESULTADOS: Observaram-se predominância do sexo feminino, idade maior ou igual a 50 anos, performance status maior ou igual a 70, e as classes mais prevalentes,de acordo com a classificação RPA, foram a V e VI. Em 72,9% dos pacientes foi realizada ressecção parcial da lesão e em 27,1%, ressecção subtotal ou total. Quimioterapia foi administrada em47,9% dos pacientes e a dose de radioterapia foi de 50û60 Gy em 72,9% dos casos. A sobrevida global mediana observada foi de 52 semanas. CONCLUSÃO: Os dados obtidos mostram que a sobrevida global de pacientes portadores de glioblastoma foi semelhante aos resultados encontrados na literatura e dependente de fatores como a adição de quimioterapia, dose de radioterapia eíndice de Karnofsky.

OBJECTIVE: To identify prognostic factors and evaluate the clinicaloutcome of patients with glioblastoma treated with surgery and radiotherapy combined or not with chemotherapy. MATERIAL AND METHOD: In this retrospective study, 48 patients with glioblastoma were treated between 1997 and 2007. All patients wereclassified according the recursive partitioning analysis (RPA) criteria.RESULTS: The majority of patients were female, with 50 years of age or above. Performance status of 70 or greater were found in 70.8% of cases, and RPA classes V and VI prevailed. Seventy-two percent of patients were submitted to partial resection and 27.1% to total or subtotal resection. Chemotherapy wasadministered in 47.9% of patients and doses between 50 and 60 Gy were delivered in 72.9%. The median overall survival was 52 weeks. CONCLUSION: Our data show an overall survival that approaches the related in others reports and were dependent of factors such as chemotherapy, dose of radiation and Karnofsky performance status.

Tectal glioblastoma

Brain stem gliomas are a heterogeneous group of neoplasms arising mostly in paediatric patients. Tectal plate gliomas represent a particular type of brain stem tumours usually with a benign, indolent clinical course, presenting with signs of raised intracranial hipertension due to supra-tentorialhydrocephalous caused by aqueductal stenosis. Seldom high-grade lesions arise in this location with tremendous therapeutic implications. When a malignant tumour is clinically and radiographically suspected a biopsy should be performed to obtain histhological confirmation. Treatment is then planned in a case-by-case basis. We present the case of a glioblastoma of the tectal plate in a 22 years-old woman operated upon by a supracerebellar-infratentorial approach.

Os gliomas do tronco cerebral são um grupo heterogêneo de neoplasias que acometem habitualmente crianças. Os gliomas da placa quadrigeminal representam um tipo particular de tumores do tronco cerebral, habitualmente com um curso benigno e indolente, surgindo com sinais de hipertensão intracraniana devido a hidrocefalia supra-tentorial provocada por compressão do aqueduto cerebral. Raramente surgem lesões de alto grau nesta região, mas as implicações terapêuticas são tremendas. Quando existe suspeita clínica e imagiológica de que se trata de lesão maligna, esta deve ser biopsada para se obter confirmação histológica. O tratamento deve então ser planejado caso a caso. Apresentamos o caso de glioblastoma da placa quadrigeminal em uma paciente de 22 anos intervencionado por via supracerebelar-infratentorial.

Simultaneous occurrence of glioblastoma at mirror image site in siblings.

Two brothers (eight and ten years) presented at the same time with features of raised intracranial pressure and found to have glioblastoma multiforme at mirror image site in parieto-occipital lobe.

Expression of cytokeratins in gliomas.

Metastatic carcinoma, which is a common malignant tumor seen in the central nervous system is often difficult to distinguish from glioblastoma multiforme. In general, neoplastic cells maintain fidelity in the expression of parent cell intermediate filament and immunohistochemistry remains the mainstay in diagnosis. A panel consisting of GFAP (usually positive for astrocytic tumors) and cytokeratin (usually positive for metastatic carcinomas) is most commonly used for this purpose. However, co-expression of two or more classes of intermediate filament proteins by neoplasms is a widespread phenomenon and there are reports of glial neoplasms expressing keratin markers. Our aims and objectives were to analyse the expression of both cytokeratin and GFAP in different glial tumors and metastatic carcinomas. Cases were collected for a period of two years. All the cases were diagnosed as primary or metastatic intracranial tumors. Formalin-fixed paraffin-embedded thin sections were taken on egg-albumin coated slides and immunostaining with GFAP and polyclonal cytokeratin was done. Forty-five tumors were analysed, including 35 glial neoplasms and 10 metastatic carcinomas of which 7 of the 32 astrocytic neoplasms (22%) showed focal immunoreactivity with pancytokeratin. All of the glial tumors but none of the metastatic carcinomas were positive with GFAP. So our conclusion was that co-expression of GFAP and CK is a fairly common phenomenon, especially in case of undifferentiated and high grade gliomas and this must be kept in mind while differentiating these cases from metastatic carcinoma, as CK positivity does not rule out the diagnosis of a glial neoplasm. Further studies with an expanded panel of CK is most useful for this.

Isolated Richter's syndrome in central nervous system: case report

Linfoma não Hodgkin difuso de grandes células em paciente portador de leucemia linfóide crônica (LLC), ou síndrome de Richter, é complicação rara e grave nesta leucemia. Síndrome de Richter isolada no sistema nervoso central é muito rara, tendo sido encontrados apenas 12 casos descritos. Descrevemos paciente de 74 anos, que apresentou linfoma não Hodgkin difuso de grandes células em região frontal direita, simulando glioblastoma multiforme.