Consenso chileno para el diagnóstico y tratamiento de gliomas del adulto / Diagnosis and treatment of glioma in adult: Chilean consensus

Resumen Los tumores cerebrales se caracterizan por su gran morbilidad y mortalidad. La gran mayoría corresponde a tumores secundarios (metástasis). Dentro de los tumores primarios del sistema nervioso central, los gliomas corresponden al 30% de éstos. En EEUU, entre el 2007-2011, se estima una incidencia aproximada de 21,4 casos por 100.000 habitantes. Los recientes avances en la comprensión molecular de la biología de estos tumores han permitido mejorar sustancialmente su clasificación, posibilitando realizar un mejor correlato con los desenlaces clínicos y el pronóstico. En esta línea, hoy en día es posible estratificar a los pacientes por riesgo y entregar tratamientos capaces de prolongar la sobrevida global entre 5-7 años, para los gliomas grado II y III. El presente consenso, elaborado por un panel multidisciplinario de expertos de diversas sociedades científicas chilenas y, por tanto, de todas las especialidades involucradas en el manejo médico-quirúrgico de las personas portadoras de gliomas cerebrales. A la luz de este nuevo conocimiento desarrollado al alero de la oncología molecular, esta propuesta ofrece un insumo de utilidad clínica real, que, articulado a una revisión actualizada en relación con el tratamiento y seguimiento de estos pacientes, permite entender la relevancia de estos biomarcadores en el manejo de precisión de la enfermedad. Cabe señalar que, este manuscrito emerge de la misma fuerza de trabajo, que elaboró el Protocolo Clínico de Gliomas del Adulto 2019, publicado por el Ministerio de Salud, y que ha diferencia de esta, que ofrece los detalles clínicos-operativos, como flujogramas y dosis, nuestra revisión intenta relevar los avances imagenológicos y moleculares y como estos impactan en el manejo actual de la enfermedad.

Brain tumors are characterized by high morbidity and mortality. The vast majority correspond to secondary tumors (metastasis). On the other hand, within the primary tumors of the central nervous system, gliomas correspond to 30% of these. In the US, between 2007-2011, an approximate incidence of 21.4 cases per 100,000 inhabitants was estimated. Recent advances in the molecular understanding of the biology of these tumors have made it possible to substantially improve their classification, allowing a better correlation with clinical outcomes and prognosis. Along these lines, today, it is possible to stratify patients by risk and deliver treatments capable of prolonging global survival between 5-7 years, for grade II and III gliomas. The present consensus, prepared by a multidisciplinary panel of experts from various Chilean scientific societies and, therefore, from all the specialties involved in the medical and surgical therapy. Enlightened from the molecular oncology, this proposal offers an input of clinical utility, which, together with an updated review in relation to the treatment and follow-up of these patients, allows us to understand the relevance of these biomarkers in precision disease management. It should be noted that this manuscript emerges from the same work force, which prepared the Clinical Protocol for Adult Gliomas 2019, published by the Ministry of Health, and that differs from it, which offers clinical-operative details, such as flowcharts and dose, our review attempts to reveal imaging and molecular advances and how they impact the current management of the disease.

Expression of Glutathione Peroxidases and Its Effect on Clinical Prognosis in Glioma Patients / 中国医学科学院学报

Objective To investigate the relationship between the expression of glutathione peroxidase(GPX)genes and the clinical prognosis in glioma patients,and to construct and evaluate the model for predicting the prognosis of glioma. Methods The clinical information and GPX expression of 663 patients,including 153 patients of glioblastoma(GBM)and 510 patients of low-grade glioma(LGG),were obtained from The Cancer Genome Atlas(TCGA)database.The relationship between GPX expression and patient survival was analyzed.The key GPX affecting the prognosis of glioma was screened out by single- and multi-factor Cox's proportional-hazards regression models and validated by least absolute shrinkage and selection operator(Lasso)regression.Finally,we constructed the model for predicting the prognosis of glioma with the screening results and then used concordance index and calibration curve respectively to evaluate the discrimination and calibration of model. Results Compared with those in the control group,the expression levels of GPX1,GPX3,GPX4,GPX7,and GPX8 were up-regulated in glioma patients(all P<0.001).Moreover,the expression levels of other GPX except GPX3 were higher in GBM patients than in LGG patients(all P<0.001).The Kaplan-Meier curves showed that the progression-free survival of GBM with high expression of GPX1(P=0.013)and GPX4(P=0.040),as well as the overall survival,disease-specific survival,and progression-free survival of LGG with high expression of GPX1,GPX7,and GPX8,was shortened(all P<0.001).GPX7 and GPX8 were screened out as the key factors affecting the prognosis of LGG.The results were further used to construct a nomogram model,which suggested GPX7 was the most important variable.The concordance index of the model was 0.843(95%CI=0.809-0.853),and the calibration curve showed that the predicted and actual results had good consistency. Conclusion GPX7 is an independent risk factor affecting the prognosis of LGG,and the nomogram model constructed with it can be used to predict the survival rate of LGG.

Comportamiento de los gliomas de alto grado en el servicio Neurocirugía de Matanzas. 2017-2019 / Behavior of high-grade gliomas at Matanzas Neurosurgery service. 2017-2019

RESUMEN Introducción: el astrocitoma anaplásico y el glioblastoma multiforme son las formas más agresivas de glioma maligno. Existen avances en radioterapia, quimioterapia y tratamientos de resección quirúrgica agresiva. Esto último incluye métodos como los de tomografía de coherencia óptica, cirugía guiada por fluorescencia, craneotomía de vigilia, terapia térmica intersticial con láser para la ablación por glioblastoma multiforme, microscopía intraoperatoria confocal y espectrometría de masas intraoperatoria, pero a pesar de todo ello el pronóstico resulta sombrío. Objetivo: determinar el comportamiento de los gliomas de alto grado en el Servicio de Neurocirugía de la provincia Matanzas. Materiales y métodos: estudio observacional, descriptivo, transversal, con los pacientes diagnosticados de gliomas de alto grado, en el Servicio Neurocirugía, de la provincia Matanzas, en el período de 1ero de enero del 2017 a 1ero de enero del 2019, para un total de 40 casos. Resultados: la edad media de las lesiones fue de 52 años, la cefalea fue el síntoma predominante, con el 72,2 %. La sintomatología se presentó con una evolución de menos de un mes. Conclusiones: en el 62 % predominaron los gliomas frontales y la variedad histológica glioblastoma multiforme. La excéresis subtotal se aplicó en la mayor cantidad de cirugías, la calidad de vida al egreso fue superior que al ingreso (AU).

ABSTRACT Introduction. Anaplastic astrocytoma (AA) and Glioblastoma multiforme (GBM) are the most aggressive forms of malignant glioma. Despite advances in radiotherapy, chemotherapy and aggressive surgical resection treatments, such as optical coherence tomography, fluorescence-guided surgery, waking craniotomy, laser interstitial thermal therapy for GBM ablation, intraoperative confocal microscopy and intraoperative mass spectrometry, the prognosis remains bleak. Objective: to determine the behavior of high grade gliomas in the Neurosurgery Service of the province of Matanzas. Materials and methods: cross-sectional, descriptive, observational study with patients diagnosed with high-grade gliomas in the Neurosurgery Service of the province of Matanzas, in the period from January 1, 2017 to January 1, 2019, for a total of 40 cases. Results: the average age of the lesionated patients was 52 years; headache was the predominant symptom, with 72.2 %; the evolution at the presentation of symptoms was less than a month. Conclusions: frontal gliomas predominated in 62 % of the cases, and predominated also glioblastoma multiforme histological variety. Subtotal excision was used in most surgeries. Life quality at discharging was higher than at the moment of admission (AU).

Role of Ectodermal-neural cortex 1 protein in human glioma progression, identification of a peptide internalized by human glioblastoma cells and development of an alternative method to generate growth curves of adherent cultures / Papel da proteína Ectodermal-neural cortex 1 (ENC1) na progressão de glioma humano, identificação de um peptídeo internalizado por células de glioblastoma humano e desenvolvimento de um método alternativo para gerar curvas de crescimento celular

Gliomas are the most common form of primary intracranial malignancy, among which astrocytomas are the most frequent. Ectodermal-cortex protein 1 (ENC 1), also known as Nuclear Restricted Protein/Brain (NRP/B), was first characterized as a protein which interacts with the cytoskeleton by binding to actin through Kelch-like domains, being related to neural fate specification during development of the nervous system. The first chapter of this thesis confirms ENC1 as a tumor suppression properties by a genomic edition approach, analyses ENC1 expression in a set of patient glioma samples and describes the correlation these data with patients survival and progression-free survival, concluding that ENC1 expression may constitute a biomarker for glioma aggressiveness. The second chapter refers to the identification and in vitro characterization of the LHTNELQ peptide, which was selected by the Phage Display method using human glioblastoma cells. This new peptide is able to be internalized by these cells and features as a new tool for the development of glioma therapeutics. The third chapter report an alternative method to generate growth curves of adherent cell cultures, which is based on the CFSE fluorescence decay over time. It is an alternative method to determine growth curves of cultured cells, with smaller variation among technical replicates than that of counting-based methods

Gliomas são a forma mais comum de malignidades primárias intracranianas, dentre os quais os astrocitomas são os mais frequentes. A proteína Ectodermal-neural cortex 1 (ENC1), também conhecida como Nuclear Restricted Protein/Brain (NRP/B), foi primeiramente caracterizada como uma proteína que interage com o citoesqueleto por meio de ligação à actina através de domínios Kelch-like, sendo relacionada com diferenciação neuronal durante o desenvolvimento do sistema nervoso. O primeiro capítulo desta tese descreve confirmação da capacidade supressora tumoral de ENC1 por abordagem de edição genômica, analisa a expressão de ENC1 em um conjunto de amostras de pacientes com gliomas e correlaciona esses dados com tempo de sobrevida geral e sobrevida livre de progressão tumoral nos pacientes, concluindo que a expressão de ENC1 pode ser utilizada como um biomarcador da agressividade do glioma. O segundo capítulo apresenta a identificação e caracterização in vitro do peptídeo LHTNELQ, que foi selecionado pela metodologia de Phage display utilizandose de células de glioblastoma humano. Este novo peptídeo é capaz de internalizar-se nestas células e figura como uma nova ferramenta para o desenvolvimento de estratégias terapêuticas para glioblastomas. No terceiro capítulo propõe-se um método alternativo para gerar curvas de crescimento celular de cultura aderente, o qual é baseado no decaimento da fluorescência do reagente CFSE ao longo do tempo. Tratase de um método alternativo para a determinação de curvas de crescimento de culturas aderentes, com menor variação entre as réplicas técnicas do que os métodos baseados em contagem das células

Importancia del diagnóstico histopatológico en el tratamiento de las lesiones del tronco cerebral en adultos / Relevance of histopathological diagnosis in the treatment of brainstem lesions in adults

Los tumores del tronco cerebral son infrecuentes en la población adulta. Las controversias surgen cuando se considera la necesidad de confirmar el diagnóstico histopatológico en esta área elocuente del cerebro, balanceando los beneficios de obtener un diagnóstico certero y las desventajas de los procedimientos invasivos. Existen escasas publicaciones acerca de su tratamiento quirúrgico en adultos, todas series pequeñas analizadas retrospectivamente. Presentamos nuestra experiencia con el propósito de contribuir al proceso de toma de decisiones. Diez de 13 pacientes fueron intervenidos. Las lesiones se clasificaron en focales (n:7), infiltrativa difusa (n:1), tectal (1) y exofítica (1). El estado neurológico según la escala Karnofsky Performance Status fue ≥ 70 en 6 casos y < 70 en 7. Las muestras fueron obtenidas mediante abordaje microquirúrgico directo o por biopsia estereotáctica. Los hallazgos histopatológicos fueron confirmados en todos los casos: astrocitoma pilocítico (n:1), glioma de bajo grado (n:1), glioblastoma (n:1), hemangioblastoma celular (n:1), subependimoma (n:1), disgerminoma (n:1), y lesiones pseudotumorales (n:4, 3 cavernomas, 1 pseudotumor inflamatorio). La amplia variedad de hallazgos patológicos en esta localización en adultos exige una precisa definición histopatológica, que no solo determina la terapéutica adecuada sino que también previene las consecuencias potencialmente catastróficoas de los tratamientos empíricos.

Brainstem tumors are uncommon beyond childhood. Controversies arise regarding the need of histological diagnosis in this eloquent area of the brain, weighting the benefits of a reliable diagnosis against the disadvantages of invasive procedures. There are scant publications about the surgical management of brainstem tumors in adults, all of them involving small retrospective cohorts. We are reporting our experience with the aim of contributing to the decision making process. Out of a series of 13 patients, 10 were approached surgically. According to Guillamo´s classification the lesions were: focal (n:7), diffuse infiltrative (n:1), tectal (n:1), and exophytic (n:1). According to the Karnofsky Performance Status scale, the neurological status was ≥ 70 in 6 cases and < 70 in 7. Histopathology was confirmed in all 10 treated cases and the samples were obtained by a direct microsurgical approach or by stereotactic biopsy. Histopathological findings were: pilocytic astrocytoma (n:1), low grade glioma (n:1), glioblastoma (n:1), cellular haemangioblastoma (n:1), subependimoma (n:1), pseudotumoral lesions (n:4; 3 cavernomas, 1 inflammatory pseudotumor), and disgerminoma (n:1). As a broad variety of pathologies could be found in this brain localization, an accurate histopathological definition can not only determine the adequate therapy, but also avoid the disastrous consequences of empiric treatments.

Magnetic resonance imaging and pathological features of a mixed glioma in a dog: case report / Aspectos patológicos e das imagens de ressonância magnética de um glioma misto em um cão: relato de caso

The aim of this report was to describe the magnetic resonance imaging (MRI) and pathological features of a canine mixed glioma. A 12-year-old boxer male dog was presented for necropsy along with data from an MRI evaluation conducted ante-mortem. The images were examined and showed a poorly demarcated prosencephalic lesion, hyperintense on T2W images, hypointense on T1W images and heterogeneously hyperintense on T2W FLAIR images. There was mild nonuniform contrast enhancement, apparent midline shift, moderate perilesional edema and marked distortion of the adjacent lateral ventricle. The brain was evaluated macroscopically, microscopically and immunohistochemically. Grossly, there was a poorly demarcated soft mass, with areas of hemorrhage, within the left parietal and temporal lobes. Histologically, there was a densely cellular mass composed of two geographically distinct populations of neoplastic cells. The first population was composed of small and round cells organized in a honeycomb pattern. The second population constituted of intermingled streams and bundles of neoplastic cells that were strongly immunolabeled for glial fibrillary acidic protein (GFAP). The diagnosis of a mixed glioma was based on MRI findings, and mainly on histological and immunohistochemical findings.(AU)

O objetivo deste relato foi descrever as características patológicas e das imagens de ressonância magnética de um glioma misto canino. Um cão de 12 anos de idade da raça Boxer foi submetido à necropsia. As imagens obtidas ante mortem por ressonância magnética foram analisadas, e nelas se observou uma lesão prosencefálica com contornos pouco definidos, sinal hiperintenso nas imagens ponderadas em T2, hipointenso nas imagens ponderadas em T1, e heterogeneamente hiperintenso em T2-FLAIR. Havia discreto realce desuniforme ao contraste, evidente desvio da linha média, edema perilesional moderado e marcada distorção do ventrículo lateral adjacente. O encéfalo foi avaliado macroscopicamente, microscopicamente e imuno-histoquimicamente. Macroscopicamente, havia uma massa pobremente demarcada, com áreas de hemorragia, nos lobos parietal e temporal esquerdos. Histologicamente, havia uma massa densamente celular, composta por duas populações de células neoplásicas distintas separadas geograficamente. A primeira população era composta por células pequenas e redondas, organizadas com aspecto de favo de mel. A segunda população era constituída por feixes entrelaçados de células neoplásicas fortemente imunomarcadas para a proteína fibrilar ácida glial (GFAP). O diagnóstico de glioma misto foi obtido com base nos achados imaginológicos e, principalmente, em suas características histológicas e imuno-histoquímicas.(AU)

Análisis de costo-efectividad de la radioterapia sola, del protocolo Stupp y del protocolo Stupp más bevacizumab para pacientes con reciente diagnóstico de glioma maligno (GBM) en Colombia / Cost-effectiveness analysis of radiotherapy alone, the Stupp protocol and the Stupp plus bevacizumab protocol for patients with a recent diagnosis of malignant glioma (GBM) in Colombia

Problema de investigación: Evaluar la RICE de la radioterapia (medida la efectividad en AVAC), el PS y el PS+BEV para el tratamiento de pacientes mayores de edad con diagnóstico reciente de GBM confirmado \r\nhistológicamente y con resección macroscópica completa. Tipo de evaluación económica: Análisis de costo\r\n-efectividad. Población objetivo: Pacientes mayores de 18 años con diagnóstico reciente de GBM confirmado histológicamente y con resección macroscópica completa. Intervención y comparadores: Comparación 1: PS comparado con radioterapia, Comparación 2: PS+BEV comparado con PS. Horizonte temporal: Se consideró un horizonte temporal de dos años, con ciclos de 1 mes de duración. Tasa de descuento: La tasa de descuento del caso base fue del 5 % y se llevaron a cabo análisis para tasas de 0 %, 3,5 %, 7 % y 12 %. Estructura del modelo: Se diseñó un modelo de Markov con tres estados de salud (estable, progresión y muerte). Fuentes de datos de efectividad y seguridad: Los datos de efectividad y seguridad fueron obtenidos de cuatro estudios clínicos multicéntricos; la información acerca de la valoración de los desenlaces fue obtenida de una ETES. Desenlaces y valoración: Las ponderaciones para la calidad de vida percibida por los pacientes en cada estado de salud fueron obtenidas de una ETES publicada previamente, y fueron utilizadas para calcular los AVAC. Costos incluidos: Fueron incluidos los costos que generan un gasto directo al SGSSS en Colombia. No se incluyeron costos de cuidadores, costos de pérdidas de productividad ni \r\ncostos de transporte. Fuentes de datos de costos: La valoración de los costos se hizo mediante el uso de \r\nSISMED y de la Circular 03 de 2015 de precios máximos en el caso de los medicamentos, y del manual tarifario ISS 2001 para el caso de los procedimientos. Resultados del caso base: La RICE del PS comparado con la radioterapia es de $ 55.410.625, lo que resulta en que no sería una alternativa costo-efectiva para el país. La RICE del PS+ BEVvs. PS ($ 1.795.371.090) comparado con tres veces el PIB per cápita hace que \r\nesta no se considere una estrategia costo-efectiva para el contexto colombiano. Análisis de sensibilidad:\r\nCuando el costo de la dosis de TMZ concomitante con radioterapia es igual a $ 136.868,6 o menos, el PS se \r\nvuelve una tecnología en salud costo-efectiva para Colombia comparado con la RT. Y solo resulta costo-efectivo a su precio actual si el umbral de disponibilidad a pagar fuera mayor a $ 50.000.000. Bajo ningún escenario de disponibilidad a pagar entre 1 y 3 PIB per cápita, el PS+BEV tendría probabilidad de ser costo-efectivo en Colombia al ser comparado con el PS. Conclusiones y discusión: Aunque las diferencias en términos de AVAC no son amplias entre las diferentes tecnologías, el uso de TMZ (es decir, el PS) es el que mejores resultados reporta frente a la radioterapia sola y frente a la inclusión del BEV al PS (aunque el uso del BEV en esta indica ción es off-label en Colombia).(AU)

Análisis de impacto presupuestal de la temozolomida para el tratamiento de pacientes adultos con diagnóstico de novo de glioma maligno en Colombia / Budgetary impact analysis of temozolomide for the treatment of adult patients with de novo diagnosis of malignant glioma in Colombia

Tecnologías evaluadas: Nueva: temozolomida. Población: Pacientes mayores de 16 años con diagnóstico reciente de glioblástoma multiforme en Colombia. Perspectiva: La perspectiva del presente AIP corresponde al tercero pagador, que en este caso es el Sistema General de Seguridad Social en Salud (SGSSS) en Colombia. Horizonte temporal: El horizonte temporal de este AIP en el caso base corresponde a un año. Adicionalmente se reportan las estimaciones del impacto presupuestal para los años 2 y 3, bajo el supuesto de la inclusión en el POS en el año 1. Costos incluidos: Costos de las tecnologías analizadas. Fuente de costos: Los precios de cada tecnología considerada fueron consultados en el manual tarifario ISS 2001 y ajustados con un +25%, +30% y +48%. Escenarios: Para este AIP no fueron diseñados escenarios de adopción debido a que la tecnología evaluada ya se utiliza en la práctica clínica colombiana, para toda la población objetivo del análisis. Resultados: El caso base involucraría una inversión total de $29.494.699.472\r\npara el año 1, $4.225.358.276,32 para el año 2 y $ 4.974.085.010,60 para el año 3. En el caso base se asuma la inclusión de la temozolomida en el POS.(AU)

Is oncolytic virotherapy a viable platform for glioma treatment?

Gliomas are devastating cancers of the nervous system with poor prognosis. Their aggressiveness produces a mortality rate rarely seen with other malignant tumours and the lack of effective treatment has left very few options. On-colytic viruses, with their long history of experimentation, have been deemed to be a key player in the future treatment of gliomas. This review will focus on the two main contenders, adenovirus and herpes simplex virus, for glioma treatment and discuss how far the field has come since its conception. The concept of each vector and the rationales behind their use will be contrasted before discussing the future of the field. Data was located by accessing the MEDLINE database using the PubMed search system. Data was selected on the basis of the insight its information provided as well as on the dependability of the experimental method used

Chordoid Glioma with Intraventricular Dissemination: A Case Report with Perfusion MR Imaging Features

Chordoid glioma is a rare low grade tumor typically located in the third ventricle. Although a chordoid glioma can arise from ventricle with tumor cells having features of ependymal differentiation, intraventricular dissemination has not been reported. Here we report a case of a patient with third ventricular chordoid glioma and intraventricular dissemination in the lateral and fourth ventricles. We described the perfusion MR imaging features of our case different from a previous report.

Glioma nasal gigante, reporte de un caso: a case report / Giant nasal glioma

Los gliomas nasales son restos de tejido neuroglial que se presentan como una masa craneofacial. Es poco frecuente y no tiene características malignas, pero local-mente es bastante agresivo. Se encuentra dentro del diagnóstico diferencial de masas congénitas de la línea media. Se presenta el caso de un recién nacido que presenta un pólipo nasal derecho y distrés respiratorio. La RNM revela una masa intranasal sin conexión intracraneal. Vía endoscópica se realiza exéresis de la masa sin complicaciones. Biopsia confirma diagnóstico de glioma.

Nasal gliomas are glial tissue residues presented as a craniofacial mass. It is rare and has no malignant features, but locally it is quite aggressive. It is included in the differential diagnosis of congenital midline masses. The case of a newborn is reported which presents a right nasal polyp and respiratory distress. The MRI reveals an intracranial mass with no intranasal connection. Endoscopic resection of the mass is done with no complications. Biopsy confirms glioma diagnosis.

Análise da marcação de células da linhagem C6 de glioma com as lectinas vegetais CPL, WGA, e Con A / cell marking analysis of C6 glioma line with plant lectins CPL, WGA and Con A

Introdução e objetivos: O glioblastoma multiforme é um glioma de alto grau que apresenta um prognóstico ruim. O diagnóstico definitivo é estabelecido pela avaliação histológica, porém este pode apresentar conflitos na classificação, com isso surge à necessidade de ferramentas que auxiliem o patologista em sua análise. Atualmente, maior ênfase tem sido dada a alterações na glicosilação, pois estão associadas a neoplasias, e a descoberta da capacidade de lectinas em reconhecer tais alterações fez destas, ferramentas aplicáveis para o diagnóstico biomédico. Dessa forma, o objetivo deste trabalho é analisar a marcação das lectinas CpL, WGA e Con A em células da linhagem C6...

Introduction and objectives: Glioblastoma multiforme is a high-grade glioma that has a poor prognosis. The definitive diagnosis is established by histological assessment. However, this can present conflicts in grading gliomas, which justifies new tools to assist the pathologist in his analysis. Currently, it is known that there are changes in glycosylation pattern of molecules associated with cancer, and the discovery of the ability of lectins to recognize these changes made these tools applicable for biomedical diagnosis. Thus, the aim of this study is to analyze the labelling of C6...

Teratoma inmaduro con gliomatosis peritoneal y embarazo / Immature teratoma with peritoneal gliomatosis in pregnancy

Presentamos el caso clínico de una paciente de 18 años sin antecedentes a destacar. Cursando las 30 semanas de embarazo se constata una tumoración abdominal que requirió tratamiento quirúrgico y se diagnosticó un teratoma inmaduro grado 3, asociado a gliomatosis peritoneal. Se realizó una cesárea al término y se instauró el tratamiento con quimioterapia presentando una recidiva a los siete meses que requirió tratamiento quirúrgico conservador y nuevo plan de quimioterapia estando actualmente libre de enfermedad y en seguimiento. Lo infrecuente de esta patología y asociada a embarazo, motivó su comunicación.

We report the case of a healthy 18-year old patient with adnexal mass diagnosed by her 30th week of pregnancy. It required surgical treatment and a grade 3 immature teratoma associated with peritoneal gliomatosis was diagnosed. A cesarean section was performed at term and subsequently chemotherapy was established. The patient presented a recurrence seven months later, which required fertility-sparing surgery and a new chemotherapy plan. The patient is currently disease-free and under surveillance. The infrequency of this disease and associated with pregnancy, led to their communication.

Gliomas: marcadores tumorais e prognóstico / Gliomas: tumors biomarkers and prognosis

Os gliomas representam 30%-40% de todas as neoplasias intracranianas e aproximadamente 50% são glioblastomas. São classificados em graus pela OMS, de acordo com sua patologia. Apresentam altas taxas de mortalidade. Existem marcadores tumorais que podem auxiliar na detecção precoce e avaliar prognóstico. Realizada revisão sobre o tema marcadores tumorais por meio do site PubMed. MGMT é uma proteína que restaura o DNA, impedindo a sua alquilação. A metilação do MGMT por meio de fenômeno epigenético impede sua transcrição inibindo sua ação, tornando o tumor suscetível a fármacos. IDH e codeleção cromossômica 1p19q são marcadores tumorais e estão associados a melhor prognóstico. As neoplasias intracranianas apresentam altas taxas de mortalidade e sua detecção precoce por meio de marcadores e o conhecimento de alterações que conferem bom prognóstico podem auxiliar no tratamento dessa doença. A análise molecular auxilia na detecção e no tratamento de tumores.

Gliomas represent 30%-40% of all intracranial tumors and approximately 50% are glioblastomas. They are classified by the WHO in degrees, according to their pathology. Have high mortality rates. There are tumor markers may help in early detection and assess prognosis. Was performed a review about the topic tumor markers through PubMed. MGMT is a protein that restores the DNA, preventing its alkylation. Methylation of MGMT through epigenetic phenomenon prevents their transcription and inhibits its action, making the tumor susceptible to drugs. IDH and chromosomal deletion 1p19q are tumor markers and are associated with better prognosis. The intracranial tumors have high rates of mortality and early detection through biomarkers and knowledge of changes that confer a good prognosis can help in treating this disease. Molecular analysis allows the detection and treatment of tumors.

Análise de sobrevida e fatores prognósticos de pacientes pediátricos com tumores cerebrais / Analysis of survival and prognostic factors of pediatric patients with brain tumor

OBJETIVOS: Realizar análise de sobrevida e avaliar, através de análise multivariada, a influência de diversas variáveis na sobrevida, definindo fatores prognósticos de pacientes pediátricos com tumores do sistema nervoso central (SNC) tratados em um único centro. MÉTODOS: Analisamos, retrospectivamente, a sobrevida de 103 crianças portadoras de tumores cerebrais primários, diagnosticadas consecutivamente no período entre janeiro de 2000 e dezembro de 2006. Análise multivariada de fatores influenciando a sobrevida global por regressão de Cox foi usada para definir possíveis fatores prognósticos. RESULTADOS: A mediana e a média de idade foram de 7,2 e 7,6 anos. Houve predominância do sexo masculino (relação 1,22:1). A maioria dos pacientes tinha meduloblastoma ou tumores neuroectodérmicos primitivos (PNET, 38 por cento) ou astrocitomas de baixo grau (18 por cento). As topografias mais comuns foram cerebelar (49 por cento) e tronco cerebral (21 por cento). A sobrevida, 5 anos após o diagnóstico, foi de 84 por cento para astrocitomas de baixo grau e 51 por cento para meduloblastomas e PNET. Fatores prognósticos para a sobrevida global foram histopatológico (astrocitomas de alto grau e ependimomas, razão de risco entre 3,7 e 3,9), cirurgia (razão de risco 0,5 para tumores completamente ressecados) e radioterapia (razão de risco 0,5 para pacientes que receberam radioterapia). CONCLUSÕES: A sobrevida global de pacientes pediátricos com tumores cerebrais neste estudo é comparável àquela dos registros populacionais dos Estados Unidos e Europa. Os fatores de prognóstico definidos para sobrevida global também se assemelham àqueles previamente publicados.

OBJECTIVES: To estimate survival and evaluate prognostic factors of pediatric patients with central nervous system (CNS) tumors treated in a single center. METHODS: Retrospective analysis of survival of 103 children with primary brain tumors diagnosed consecutively from January 2000 to December 2006. Cox regression was used for multivariate analysis of factors that affect overall survival to define possible prognostic factors. RESULTS: Median and mean ages were 7.2 and 7.6 years. There was a male predominance (1.22:1). Most patients had medulloblastomas or primitive neuroectodermal tumors (PNET, 38 percent), or low-grade astrocytomas (18 percent). The anatomic site of most tumors was the cerebellum (49 percent) and the brain stem (21 percent). Five-year survival after diagnosis was 84 percent for low-grade astrocytomas and 51 percent for medulloblastomas and PNET. Prognostic factors for overall survival were histopathological type (high-grade astrocytomas and ependymomas; hazard ratio = 3.7 to 3.9), surgery (hazard ratio of 0.5 for completely resected tumors) and radiotherapy (hazard ratio of 0.5 for patients who underwent radiotherapy). CONCLUSIONS: Overall survival of pediatric patients with brain tumors in this study was similar to that found in populations of the United States and Europe. The prognostic factors defined for overall survival are also similar to those published in previous studies.