Evaluation of the prognostic potential of EGFL7 in pilocytic astrocytomas.

Pilocytic astrocytoma (PA) is the most frequent solid neoplasm in childhood. It has a good 5-year overall survival (90% in childhood and 52% in adults). However, up to 20% of patients experience residual tumor growth, recurrence, and death. Although the main genetic alteration of PAs, including KIAA1549:BRAF fusion, involves chromosome 7q34, we previously found frequent loss in chr9q34.3 locus in a small subset of these tumors. Among the genes present in this locus, EGFL7 is related to poor prognosis in several tumor types. In this study, we aimed to assess EGFL7 expression through immunohistochemistry, and to evaluate its prognostic value in a series of 64 clinically and molecularly well-characterized pilocytic astrocytomas. We found high expression of EGFL7 in 71.9% of patients. Low EGFL7 expression was associated with older patients, the mean age mainly older than 11 years (P = 0.027). EGFL7 expression was not associated with presence of KIAA1549:BRAF fusion, BRAF mutation, FGFR1 mutation, nor FGFR1 duplication. Moreover, high EGFL7 expression was associated with high FGFR1 (P = 0.037) and 5'-deoxy-5'-methyltioadenosine phosphorylase (MTAP) (P = 0.005) expression, and with unfavorable outcome of patients (P = 0.047). Multivariate analysis revealed low EGFL7 expression related to older patients and high EGFL7 expression related to retained expression of MTAP. In addition, we found a borderline significance of unfavorable outcome and high EGFL7 expression. Finally, EGFL7 expression was not associated with overall or event-free survival of PA patients. Our findings point to EGFL7 expression as a novel candidate prognostic marker in PA, which should be further investigated.

Evaluation of the melting temperature of TaqMan probes as a genotyping method for IDH1, IDH2, and H3F3A in pediatric astrocytomas / Evaluación del método de temperatura de disociación de sondas TaqMan para la genotipificación de IDH1, IDH2 y H3F3A en astrocitomas pediátricos

Abstract Background: Astrocytomas are cancer tumors of the central nervous system and represent the most common type of solid tumors during human childhood. In 2016, the World Health Organization established a molecular classification system to regroup tumor entities to achieve a more accurate diagnosis and a better clinical decision-making and selection of treatment in patients with these types of tumors. Methods: We evaluated a genotyping assay for rapid and cost-effective mutation detection in astrocytomas using TaqMan probes in an asymmetric polymerase chain reaction (PCR) assay. Results: Four diffuse astrocytomas (Grade II), three anaplastic astrocytomas (Grade III), and four glioblastomas (Grade IV) were sequenced, and all of them displayed the wild-type (WT) sequence. We tried to set up this melting analysis for the genotyping of pediatric astrocytomas by identifying the specific melting temperatures of the TaqMan probes due to the presence of the WT sequences in the isocitrate dehydrogenase 1 and 2 (IDH1 and IDH2) and H3.3 histone A genes (H3F3A). We used an IDH1-TaqMan probe to identify the WT status of IDH1 in two different WT deoxyribonucleic acid (DNA) templates (pilocytic and diffuse astrocytoma) and obtained four melting temperature values ranged from 65.6 to 92.2°C. Furthermore, only four out of 29 reactions displayed amplification of the DNA template. Sanger sequencing was faster and more reliable to detect the gene status in all the sequenced samples. Conclusions: We conclude that conventional Sanger sequencing remains the gold standard for the genotyping of pediatric astrocytomas.

Resumen Introducción: Los astrocitomas son un tipo de cáncer que afecta al sistema nervioso central y representan el tumor sólido más común durante la infancia. En el año 2016, la Organización Mundial de la Salud estableció un sistema de clasificación molecular para reagrupar tumores con identidades genéticas similares y lograr un diagnóstico más preciso, lo que lleva a tomar las decisiones clínicas idóneas al elegir el tratamiento de pacientes con este tipo de tumores. Métodos: Se evaluó un protocolo que involucra el uso de sondas TaqMan en un ensayo de reacción en cadena de la polimerasa asimétrica para la detección de mutaciones en astrocitomas. Se secuenciaron cuatro astrocitomas difusos (Grado II), tres astrocitomas anaplásicos (Grado III) y cuatro glioblastomas (Grado IV). Se intentó establecer las condiciones del análisis para la genotipificación de los astrocitomas pediátricos mediante la identificación de las temperaturas de disociación específicas de las sondas TaqMan producidas por la prescencia de las secuancias WT en los genes isocitrato deshidrogenasa 1 y 2 (IDH1, IDH2) y H3.3 histona A (H3F3A). Resultados: Los astrocitomas mostraron la secuencia wild type (WT) (silvestre) de los genes. Se utilizó una sonda TaqMan IDH1 para identificar el estado de este gen en dos templados WT de DNA (astrocitoma pilocítico y difuso) y se obtuvieron cuatro valores de temperatura de disociación (65.6-92.2 °C). Solo cuatro de las 29 reacciones mostraron amplificación de DNA. La secuenciación de Sanger fue más rápida y confiable para detectar el estado de los genes en todas las muestras. Conclusiones: La secuenciación de Sanger sigue siendo la técnica más práctica para la genotipificación de astrocitomas pediátricos.

Evaluation of the melting temperature of TaqMan probes as a genotyping method for IDH1, IDH2, and H3F3A in pediatric astrocytomas.

Background: Astrocytomas are cancer tumors of the central nervous system and represent the most common type of solid tumors during human childhood. In 2016, the World Health Organization established a molecular classification system to regroup tumor entities to achieve a more accurate diagnosis and a better clinical decision-making and selection of treatment in patients with these types of tumors. Methods: We evaluated a genotyping assay for rapid and cost-effective mutation detection in astrocytomas using TaqMan probes in an asymmetric polymerase chain reaction (PCR) assay. Results: Four diffuse astrocytomas (Grade II), three anaplastic astrocytomas (Grade III), and four glioblastomas (Grade IV) were sequenced, and all of them displayed the wild-type (WT) sequence. We tried to set up this melting analysis for the genotyping of pediatric astrocytomas by identifying the specific melting temperatures of the TaqMan probes due to the presence of the WT sequences in the isocitrate dehydrogenase 1 and 2 (IDH1 and IDH2) and H3.3 histone A genes (H3F3A). We used an IDH1-TaqMan probe to identify the WT status of IDH1 in two different WT deoxyribonucleic acid (DNA) templates (pilocytic and diffuse astrocytoma) and obtained four melting temperature values ranged from 65.6 to 92.2°C. Furthermore, only four out of 29 reactions displayed amplification of the DNA template. Sanger sequencing was faster and more reliable to detect the gene status in all the sequenced samples. Conclusions: We conclude that conventional Sanger sequencing remains the gold standard for the genotyping of pediatric astrocytomas.

Introducción: Los astrocitomas son un tipo de cáncer que afecta al sistema nervioso central y representan el tumor sólido más común durante la infancia. En el año 2016, la Organización Mundial de la Salud estableció un sistema de clasificación molecular para reagrupar tumores con identidades genéticas similares y lograr un diagnóstico más preciso, lo que lleva a tomar las decisiones clínicas idóneas al elegir el tratamiento de pacientes con este tipo de tumores. Métodos: Se evaluó un protocolo que involucra el uso de sondas TaqMan en un ensayo de reacción en cadena de la polimerasa asimétrica para la detección de mutaciones en astrocitomas. Se secuenciaron cuatro astrocitomas difusos (Grado II), tres astrocitomas anaplásicos (Grado III) y cuatro glioblastomas (Grado IV). Se intentó establecer las condiciones del análisis para la genotipificación de los astrocitomas pediátricos mediante la identificación de las temperaturas de disociación específicas de las sondas TaqMan producidas por la prescencia de las secuancias WT en los genes isocitrato deshidrogenasa 1 y 2 (IDH1, IDH2) y H3.3 histona A (H3F3A). Resultados: Los astrocitomas mostraron la secuencia wild type (WT) (silvestre) de los genes. Se utilizó una sonda TaqMan IDH1 para identificar el estado de este gen en dos templados WT de DNA (astrocitoma pilocítico y difuso) y se obtuvieron cuatro valores de temperatura de disociación (65.6-92.2 °C). Solo cuatro de las 29 reacciones mostraron amplificación de DNA. La secuenciación de Sanger fue más rápida y confiable para detectar el estado de los genes en todas las muestras. Conclusiones: La secuenciación de Sanger sigue siendo la técnica más práctica para la genotipificación de astrocitomas pediátricos.

Pilocytic Astrocytoma: A Review of General, Clinical, and Molecular Characteristics.

Pilocytic astrocytomas are the primary tumors most frequently found in children and adolescents, accounting for approximately 15.6% of all brain tumors and 5.4% of all gliomas. They are mostly found in infratentorial structures such as the cerebellum and in midline cerebral structures such as the optic nerve, hypothalamus, and brain stem. The present study aimed to list the main characteristics about this tumor, to better understand the diagnosis and treatment of these patients, and was conducted on search of the published studies available in NCBI, PubMed, MEDLINE, Scielo, and Google Scholar. It was possible to define the main histologic findings observed in these cases, such as mitoses, necrosis, and Rosenthal fibers. We described the locations usually most affected by tumor development, and this was associated with the most frequent clinical features. The comparison between the molecular diagnostic methods showed great use of fluorescent in situ hybridization, polymerase chain reaction (PCR), and reverse transcriptase-PCR, important techniques for the detection of BRAF V600E mutation and BRAF-KIAA1549 fusion, characteristic molecular alterations in pilocytic astrocytomas.

Uso de nimotuzumab en paciente con astrocitoma anaplásico / Nimotuzumab in the treatment of a patient with anaplastic astrocytoma

Introducción: Nimotuzumab es una inmunoglobina de isotipo IgG1, obtenido por tecnología de ADN recombinante. La expectativa de vida de niños con tumores cerebrales recurrentes, refractarios a tratamientos a la cirugía, la radioterapia y la quimioterapia es de un mes aproximadamente. Con este tratamiento la supervivencia alcanza 44,5 meses. Objetivos: Presentar el caso clínico de un paciente con diagnóstico de Astrocitoma anaplásico que recibió tratamiento oncoespecífico concurrente con Nimotuzumab. Presentación de caso: Se realizó la descripción del diagnóstico, tratamiento y evolución de un paciente de 31 años de edad que fue diagnosticado con una neoplasia del sistema nervioso central. (Astrocitoma anaplásico). Recibió la combinación terapéutica de cirugía, radioterapia y anticuerpos monoclonales, lográndose una sobrevida de 39 meses. Conclusiones: La adición del anticuerpo monoclonal al tratamiento estándar de los tumores cerebrales aumentó la sobrevida del paciente, convirtiéndose en una alternativa terapéutica segura, ventajosa y factible como parte del tratamiento convencional en las condiciones asistenciales(AU)

Introduction: Nimotuzumab is an IgG1 isotype immunoglobin, obtained by recombinant DNA technology. Life expectancy is approximately one month in children with recurrent brain tumors, refractory to treatments to surgery, radiotherapy and chemotherapy. Survival reaches 44.5 months when using Nimotuzumab. Objectives: To report the clinical case of a patient diagnosed with anaplastic astrocytoma who received concurrent oncospecific treatment with Nimotuzumab. Case report: This paper describes the diagnosis, treatment and evolution of a 31-year-old male patient with neoplasm of the central nervous system (Anaplastic astrocytoma). He received the therapeutic combination of surgery, radiotherapy and monoclonal antibodies, achieving a survival of 39 months. Conclusions: The adding the monoclonal antibody to the standard treatment of brain tumors increased patient survival, making it a safe, advantageous and feasible therapeutic alternative as part of conventional treatment in healthcare conditions(AU)

Astrocitoma medular de alto grado no infiltrante / Astrocitoma medular não infiltrativo. Apresentação do caso / Non-infiltrating medullary astrocytoma. Case presentation

Se presentó el caso de una paciente femenina de 20 años de edad que acudió al servicio de Neurocirugía por presentar dolor en la región lumbar. Al interrogatorio la paciente refirió tener constipación, retención urinaria y dolor lumbar. En los exámenes complementarios lo más significativo fue la resonancia magnética nuclear que mostró una imagen hiperdensa a nivel de D12-L5. Se realizó laminectomía de D12-L5, durotomía y extracción tumoral, fijación transpedicular con tornillos ubicados en T12-L1-L3-L4 con barras de titanio, se obtuvo un diagnóstico de astrocitoma grado II-III. Al analizar los diferentes aspectos del caso en cuestión, los investigadores concluyen que con la conducta terapéutica tomada con este caso se sientan las bases para un correcto manejo de posteriores pacientes(AU)

The case of a 20-year-old female patient who went to the Neurosurgery department for presenting pain in the lumbar region was presented. At the interrogation, the patient reported having constipation, urinary retention and low back pain. In the complementary tests, the most significant was nuclear magnetic resonance, which showed a hyperdense image at the level of D12-L5. Laminectomy of D12-L5, durotomy and tumor extraction, transpedicular fixation with screws located in T12-L1-L3-L4 with titanium bars was performed, a diagnosis of grade II-III astrocytoma was obtained. When analyzing the different aspects of the case in question, the researchers conclude that with the therapeutic behavior taken with this case, the basis is laid for the correct management of subsequent patients(AU)

Foi apresentado o caso de uma paciente de 20 anos que foi ao departamento de Neurocirurgia por apresentar dor na região lombar. No interrogatório, o paciente relatou constipação, retenção urinária e lombalgia. Nos testes complementares, o mais significativo foi a ressonância magnética nuclear, que mostrou uma imagem hiperdensa ao nível de D12-L5. Foi realizada laminectomia de D12-L5, durotomia e extração de tumores, fixação transpedicular com parafusos localizados em T12-L1-L3-L4 com barras de titânio e foi obtido o diagnóstico de astrocitoma grau II-III. Ao analisar os diferentes aspectos do caso em questão, os pesquisadores concluem que, com o comportamento terapêutico adotado neste caso, é lançada a base para o manejo correto dos pacientes subsequentes)AU=

Síndrome diencefálico en un niño con desnutrición crónica e hiperactividad / Diencephalic syndrome in a child with chronic malnutrition and hyperactivity

El síndrome diencefálico es una causa infrecuente de desnutrición. Se produce por la disfunción del hipotálamo y está asociado a tumores del encéfalo. Los pacientes presentan una grave y progresiva pérdida de peso, aunque el apetito y la ingesta calórica son, por lo general, adecuados. Característicamente, los síntomas neurológicos son tardíos, lo que retrasa la sospecha diagnóstica. Se presenta a un paciente de 2 años y 6 meses de edad con desnutrición crónica grado II, derivado con diagnóstico presuntivo de enfermedad celíaca con mala adherencia y fracaso del tratamiento. Durante la internación, se arribó al diagnóstico de síndrome diencefálico secundario a un astrocitoma pilocítico grado I.

Diencephalic syndrome is an infrequent cause of malnutrition. It is produced by a malfunctioning hypothalamus, and it is related to encephalic tumors. Patients present a serious and progressive weight loss although the appetite and calorie intake are, usually, adequate. Neurological symptoms typically have a late appearance, delaying diagnostic suspicion. We present a patient aged 2 years and a half with grade II chronic malnutrition, referred with presumptive diagnosis of celiac disease, with poor adherence and treatment failure. During hospitalization, diagnosis of diencephalic syndrome secondary to grade I pilocytic astrocytoma was reached.

[Diencephalic syndrome in a child with chronic malnutrition and hyperactivity]. / Síndrome diencefálico en un niño con desnutrición crónica e hiperactividad.

Diencephalic syndrome is an infrequent cause of malnutrition. It is produced by a malfunctioning hypothalamus, and it is related to encephalic tumors. Patients present a serious and progressive weight loss although the appetite and calorie intake are, usually, adequate. Neurological symptoms typically have a late appearance, delaying diagnostic suspicion. We present a patient aged 2 years and a half with grade II chronic malnutrition, referred with presumptive diagnosis of celiac disease, with poor adherence and treatment failure. During hospitalization, diagnosis of diencephalic syndrome secondary to grade I pilocytic astrocytoma was reached.

El síndrome diencefálico es una causa infrecuente de desnutrición. Se produce por la disfunción del hipotálamo y está asociado a tumores del encéfalo. Los pacientes presentan una grave y progresiva pérdida de peso, aunque el apetito y la ingesta calórica son, por lo general, adecuados. Característicamente, los síntomas neurológicos son tardíos, lo que retrasa la sospecha diagnóstica. Se presenta a un paciente de 2 años y 6 meses de edad con desnutrición crónica grado II, derivado con diagnóstico presuntivo de enfermedad celíaca con mala adherencia y fracaso del tratamiento. Durante la internación, se arribó al diagnóstico de síndrome diencefálico secundario a un astrocitoma pilocítico grado I.

Copy Number Profiling of Brazilian Astrocytomas.

Copy number alterations (CNA) are one of the driving mechanisms of glioma tumorigenesis, and are currently used as important biomarkers in the routine setting. Therefore, we performed CNA profiling of 65 astrocytomas of distinct malignant grades (WHO grade I-IV) of Brazilian origin, using array-CGH and microsatellite instability analysis (MSI), and investigated their correlation with TERT and IDH1 mutational status and clinico-pathological features. Furthermore, in silico analysis using the Oncomine database was performed to validate our findings and extend the findings to gene expression level. We found that the number of genomic alterations increases in accordance with glioma grade. In glioblastomas (GBM), the most common alterations were gene amplifications (PDGFRA, KIT, KDR, EGFR, and MET) and deletions (CDKN2A and PTEN) Log-rank analysis correlated EGFR amplification and/or chr7 gain with better survival of the patients. MSI was observed in 11% of GBMs. A total of 69% of GBMs presented TERT mutation, whereas IDH1 mutation was most frequent in diffuse (85.7%) and anaplastic (100%) astrocytomas. The combination of 1p19q deletion and TERT and IDH1 mutational status separated tumor groups that showed distinct age of diagnosis and outcome. In silico validation pointed to less explored genes that may be worthy of future investigation, such as CDK2, DMRTA1, and MTAP Herein, using an extensive integrated analysis, we indicated potentially important genes, not extensively studied in gliomas, that could be further explored to assess their biological and clinical impact in astrocytomas.

Astrocitoma pilocítico en niños: Reporte de un caso / Pilocytic astrocytoma in children. Case Report

Se reporta el caso de un niño de 10 años que se presentó con dos semanas de enfermedad caracterizada por cefalea, vómitos, marcha tambaleante y somnolencia. El examen clínico reveló bradilalia, ataxia, hiperreflexia, nistagmo horizontal y vertical, parálisis del VI nervio craneal izquierdo y signos de frontalización. En la tomografía y resonancia cerebrales se encontró hidrocefalia obstructiva por un tumor de la fosa posterior. La anatomía patológica reveló Astrocitoma Pilocítico grado I (WHO)...

We report a 10 years old boy with 2 weeks of symptoms characterized by headache, vomiting, staggering gait and drowsiness. The initial clinical examination showed bradilalia, ataxia, hyperreflexia, horizontal and vertical nystagmus, VI left cranial nerve paresis and signs of frontalization. The brain CT and MRI showed a posterior fossa tumor causing hydrocephaly. The histhopatology revealed grade I pilocytic astrocytoma (WHO)...

Correlação entre ressonância magnética, expressão do Ki-67 e p53, e prognóstico em astrocitomas / Correlation between Magnetic Ressonance Image, Expression of Ki-67 and p53, and Prognosis in Astrocytomas

Objetivos Avaliar a correlação entre a expressão do Ki-67 e do p53 em astrocitomas graus II a IV, as características da RM e seu prognóstico. Métodos e Resultados Este é um estudo longitudinal retrospectivo do tipo coorte histórico que avaliou 45 pacientes. O prognóstico foi avaliado mediante revisão dos prontuários. A avaliação imuno-histoquímica foi realizada no Serviço de Patologia do HB. A avaliação das imagens de RMfoi feita no Serviço de Radiologia. A sobrevidamédia dos pacientes foi de 581,4 dias no grau IV; 1.704,8 dias no grau III; e 1.044,8 dias no grau II. Para cada grau, os seguintes percentuais de células positivas de Ki-67 e p53 tiveram respectivamente: 97,8 e 80% de positividade no grau IV; 72,7 e 73,4% de positividade no grau III; e 70 e 80% de positividade no grau II. Observou-se diferença estatística na expressão de Ki-67 entre os graus III e IV (P » 0,014). A RM mostrou especificidade de 64% e sensibilidade de 100% para a presença de necrose tumoral. Conclusão Concluímos que a análise da positividade do Ki-67 e do p53 não permite sua correlação com a sobrevida dos pacientes, apesar de ambos terem sua frequência distribuída de maneira semelhante. A RM tem especificidade moderada em relação ao exame patológico.rada em relação ao exame patológico.

Objectives To evaluate the correlation between the expression of Ki -67 and p53 in astrocytomas grades II to IV, the characteristics of MRI and its prognosis. Methods This was a retrospective longitudinal cohort study of its kind that evaluated 45 patients. Prognosis was assessed by review of medical records. The immunohistochemical evaluation was performed at the Pathology Service of HB. The evaluation of MRI images was taken in the Radiology Department. The median survival of patients was 581.4 days in grade IV; 1704.8 days in grade III; 1044.8 days in grade II. For each grade, the following percentages of cells positive for Ki -67 and p53 , respectively : 97.8% and 80% had a positive grade IV ; 72.7% and 73.4% positivity in grade III ; 70% and 80% in stage II positivity . There was statistical difference in Ki -67 between grades III and IV (P » 0.014).MRI showed 64% specificity and 100% sensitivity for the presence of tumor necrosis Conclusion We conclude that the analysis of the positivity of Ki -67 and p53 expression does not allow correlation with patient survival, although both their frequency distributed similarly. MRI hasmoderate specificity in relation to pathological examination.

Astrocitoma pilocítico da região selar/suprasselar determinando manifestações endócrinas / Pilocytic astrocytoma of sellar/suprasellar region determining endocrine manifestations

Pilocytic astrocytoma (PA) is a grade I glial neoplasm arising mainly in the cerebellum of children. Herein, the authors report a case of PA in a 21 year-old male patient, who presented headache, vomiting and delayed pubertal development. Serum level of cortisol and testosterone corresponded to 32.8 ug/dl and 0.19 ng/ml, respectively. The computed tomography/magnetic resonance (CT/RM) imaging showed an expansive process compromising suprasellar/hypothalamic region and determining hydrocephalus. The patient underwent resection of the process. Histological evaluation revealed a glial neoplasm constituted by loose glial tissue, small microcysts, areas of dense piloid tissue and Rosenthal fibers. The neoplastic cells were immunoreactive for glial fibrillary acidic protein (GFAP) and negative for chromogranin and synaptophysin. The diagnosis of PA was then established.

O astrocitoma pilocítico (AP) é uma neoplasia glial grau I encontrada principalmente no cerebelo de crianças. Os autores relatam um caso de AP em paciente masculino, 21 anos de idade, que apresenta cefaleia, vômitos e retardo do desenvolvimento puberal. Os níveis séricos de cortisol e testesterona corresponderam a 32,8 ug/dl e 0,19 ng/ml. A tomografia computadorizada/ressonância magnética (TC/RM) identificaram um processo expansivo que comprometia a região suprasselar/hipotalâmica e determinava hidrocefalia.O paciente foi submetido à ressecção do processo. À microscopia, foi identificada uma neoplasia glial constituída por tecido glial frouxo, pequenos microcistos, áreas de tecido piloide denso e fibras de Rosenthal. As células neoplásicas foram imunopositivas para glial fibrillary acidic protein (GFAP) e negativas para cromogranina e sinaptofisina. O diagnóstico de AP foi, então, estabelecido.

Subependymal giant cell astrocytoma associated with tuberous sclerosis presenting with intratumoral bleeding. Case report and review of literature.

The authors describe a rare case of subependymal giant cell astrocytoma (SEGA) associated with tuberous sclerosis complex that presented with intratumoral bleeding with extension to the ventricles. The literature regarding intracranial hemorrhage of SEGA is reviewed and only five cases have been reported in the literature. We have not identified a histological feature associated with bleeding.

Astrocitoma pilomixoide de fossa posterior em criança: relato de caso / Pilomyxoid astrocytoma of the posterior fossa in children: case report

Descrever um caso de astrocitoma pilomixoide, um tumor do sistema nervoso central raro, descrito e classificado recentemente diante de sintomatologia e achados pouco específicos. Análise e descrição de um caso de astrocitoma pilomixoide submetido a tratamento cirúrgico no Instituto José Frota, Fortaleza, Ceará. Paciente evolui com regressão importante dos sintomas, sem indícios de recidiva tumoral. O diagnóstico e a classificação correta dos tumores cerebrais são de extrema importância clínica, pois modificam de forma impactante o prognóstico, assim como a abordagem terapêutica.

To describe a pilomyxoid astrocytoma case, a tumor of the central nervous system rare recently described and classified in the face of a specific symptomatology and little found. Analysis and description of a case of pilomyxoid astrocytoma underwent surgical treatment at the José Frota Institute in Fortaleza, Ceará. Patient developed a significant decrease of symptoms without evidence of tumor relapse. The diagnosis and the correct classification of cerebral tumors are of extreme clinical importance, because it changes so as impacting the prognosis as well as therapeutic approach.

Unusual clinical presentation of astrocytoma in an adult.

BACKGROUND: Brainstem gliomas have a varied biological behavior based upon histopathological characteristics, localization and related diseases. The objective of this study is to present the clinical case of a grade II (diffuse) astrocytoma in an adult with an atypical clinical presentation including initial hiccup and dysphagia in a patient without evidence of clinical primary gastroenterology pathology. CLINICAL CASE: We describe the case of a 51-year-old male with a 4-year history of hiccup, oropharyngeal dysphagia and neurological clinical elements of tongue fasciculations, quadriparesis, generalized hyperreflexia due to astrocytoma with atypical localization in the floor of the fourth ventricle and histopathological diagnosis of low-grade diffuse glioma. CONCLUSIONS: Hiccup is a nonspecific symptom infrequently associated with neurological disorders. Adequate semiology of oropharyngeal dysphagia would help us to identify neurological entities such as in the case presented here. Additionally, with signs of upper and lower motor neuron we should suspect the possibility of central nervous system abnormalities of functional and organic etiology. Computed axial tomography and magnetic resonance imaging studies are important elements for diagnosis.

Intellectual impairment after treatment for medulloblastoma and astrocytoma in childhood: the Brazilian experience.

BACKGROUND: This study investigated the impact of posterior fossa tumors and their respective treatments, and the impact of clinical and sociodemographic variables, on the intelligence quotient (IQ) of Brazilian children. METHODS: Twenty patients took part in the study, of which 13 were diagnosed with astrocytoma (average age at evaluation 10.2 y) and 7 with medulloblastoma (average age at evaluation 9.2 y). The first subgroup was submitted exclusively to tumor resection surgery and the second subgroup underwent surgery, chemotherapy (Vincristine, Cisplatine, and Carmustine), and radiotherapy (total dose of 54 Gy). The Wechsler Intelligence Scale for Children (WISCIII) was used. RESULTS: The following statistically significant effects were identified: treatment modality on performance intelligence quotient scores (P=0.02) and processing speed index (PSI) (P=0.01); presence of hydrocephalus at diagnosis on verbal intelligence quotient (P=0.04); tumor localization on perceptual organization index (P=0.03); time interval between diagnosis and neuropsychological evaluation on PSI (P=0.05) and freedom from distraction index (P=0.03); and level of parental formal education on full scale IQ (P=0.02). CONCLUSIONS: Exposure to radiotherapy has a significant effect on processing speed and consequently on global intellectual capacity. The impact on intelligence of clinical and sociodemographic variables such as tumor localization, time interval between diagnosis and cognitive evaluation, and parental level of formal education is confirmed in the specific setting of a developing country.