Distribuição da Mortalidade Infantojuvenil por Tumores do Sistema Nervoso Central no Estado do Ceará / Distribution of Children and Adolescent Mortality by Central Nervous System Tumors in the State of Ceará / Distribución de la Mortalidad Infantil y Juvenil por Tumores del Sistema Nervioso Central en el Estado de Ceará

Introdução: Os tumores do sistema nervoso central (SNC) são um dos tipos mais comuns de tumores sólidos que acometem crianças. Apresentam heterogeneidade por compreender mais de 100 tipos histológicos definidos com base na origem da célula e outras características histopatológicas. Objetivo: Analisar a distribuição espacial da mortalidade por tumores do SNC em crianças e adolescentes no Estado do Ceará, no período de 2008-2018. Método: Estudo ecológico realizado com dados de fontes secundárias de 2008-2018 disponíveis no Sistema de Informação sobre Mortalidade (SIM). Foram calculadas as taxas brutas, o Índice de Moran Global e Local e o risco relativo para todos os óbitos. Resultados: Dos 378 óbitos, 197 eram do sexo masculino (52%), 116 apresentavam a faixa etária entre 5-9 anos (30,7%), 193 eram pardos (51%) e 354 morreram no hospital (93,6%). Os municípios que apresentaram as maiores taxas (49,94 e 99,88 óbitos/1 milhão de habitantes) foram Aracati, Barreira, Catunda, Coreaú e Fortaleza. Pelo Índice de Moran, observou-se a formação de um cluster com padrão alto-alto e significância estatística de distribuição na Mesorregião do Metropolitana de Fortaleza. O município de Pacujá apresentou o maior risco relativo (7,32) seguido do município de Catunda (6,94). Conclusão: As Regiões Norte e Noroeste do Ceará possuem taxas mais elevadas e risco maior para mortalidade por tumores do SNC

Introduction: Central nervous system (CNS) tumors are one of the most common types of solid tumors that affect children. Due to their heterogeneity, they comprise more than 100 defined histological types of cell origin-based and other histopathological characteristics. Objective: To analyze the spatial distribution of mortality by CNS tumors in children and adolescents in the State of Ceará in the period 2008-2018. Method: Ecological study, with secondary data of the period selected available in the Mortality Information System (SIM). Crude rates, Moran Global and Local index, and relative risk for all deaths were calculated. Results: Of the 378 deaths, 197 were males (52%) mostly, 116 aged between 5-9 years (30.7%), 193 brown (51%) and 354 died in the hospital (93.6%). The municipalities with the highest rates (49.94 and 99.88 deaths/1 million inhabitants) were Aracati, Barreira, Catunda, Coreaú and Fortaleza. The Moran Index showed the formation of a cluster with a high-high standard and statistical significance of distribution in the Metropolitana Fortaleza Mesoregion. The municipality of Pacujá had the highest relative risk (7.32) followed by the municipality of Catunda (6.94). Conclusion: The North and Northwest Regions of Ceará presented the higher mortality and risk rates by CNS tumors

Introducción: Los tumores del sistema nervioso central (SNC) son uno de los tipos más comunes de tumores sólidos que afectan a los niños. Presentan heterogeneidad al comprender más de 100 tipos histológicos definidos y basados en el origen celular y otras características histopatológicas. Objetivo: Analizar la distribución espacial de la mortalidad por tumores del SNC en niños y adolescentes en el Estado de Ceará en el período 2008-2018. Método: Estudio ecológico realizado con datos de fuentes secundarias de 2008-2018 disponibles en el Sistema de Información de Mortalidad (SIM). Se calcularon las tasas brutas, el Índice de Moran Global y Local y el riesgo relativo de todas las muertes. Resultados: De los 378 fallecidos, 197 eran varones (52%), 116 presentaban el grupo de edad entre 5-9 años (30,7%), 193 eran morenos (51%) y 354 murieron en el hospital (93,6%). Los municipios con mayores tasas (49,94 y 99,88 defunciones/1 millón de habitantes) fueron Aracati, Barreira, Catunda, Coreaú y Fortaleza. El Índice de Moran observó la formación de un cluster con un patrón alto-alto y significación estadística de distribución en la Mesoregión de Fortaleza Metropolitana. El municipio de Pacujá presentó el mayor riesgo relativo (7,32) seguido del municipio de Catunda (6,94). Conclusión: Las regiones del Norte y Noroeste de Ceará tienen tasas más elevadas y un mayor riesgo de mortalidad por tumores del SNC

The role of surgery in primary central nervous system lymphomas / El rol de la cirugía en los linfomas primarios del sistema nervioso central

ABSTRACT Background Primary central nervous system lymphomas (PCNSL) are infrequent. The traditional treatment of choice is chemotherapy. Complete resections have generally not been recommended, because of the risk of permanent central nervous system deficits with no proven improvement in survival. The aim of the current study was to compare survival among patients with PCNSL who underwent biopsy versus surgical resection. Methods A retrospective study was conducted on 50 patients with a confirmed diagnosis of PCNSL treated at our center from January 1994 to July 2015. Results Patients in the resection group exhibited significantly longer median survival time, relative to the biopsy group, surviving a median 31 months versus 14.5 months; p = 0.016. Conclusions In our series, patients who had surgical resection of their tumor survived a median 16.5 months longer than patients who underwent biopsy alone.

RESUMO Introducción Los linfomas primarios del sistema nervioso central (LPSNC) son infrecuentes. Tradicionalmente el tratamiento de elección es la quimioterapia. Existe un paradigma de no indicar resección, por el riesgo de déficit neurológico sin aumento de la sobrevida. El objetivo del presente estudio es comparar la sobrevida de pacientes con LPSNC sometidos a biopsia versus resección. Métodos Estudio retrospectivo que incluye 50 pacientes con diagnóstico confirmado de LPSNC tratados en nuestra Institución desde enero de 1994 hasta julio de 2015. Resultados Los pacientes del "grupo resección" mostraron una sobrevida media significativamente mayor respecto a los del "grupo biopsia"; 31 meses versus 14,5 meses respectivamente, p = 0,016. Conclusiones En nuestra serie, los pacientes que con resección quirúrgica de su tumor tuvieron una sobrevida media de 16,5 meses superior que los pacientes biopsiados.

Análise de sobrevida de pacientes portadores de tumores do sistema nervoso central / Survival analysis of patients with central nervous tumor

Objetivos: analisar a sobrevida de pacientes com diagnóstico de tumores do sistema nervoso central (SNC) em um serviço de referência em Oncologia Pediátrica, sua apresentação clínica, evolução e influência de fatores prognósticos. Métodos: estudo de coorte histórica. Foram estudados pacientes menores de 19 anos, no período de março de 2003 a dezembro de 2009. O método de Kaplan-Meier foi utilizado para estimar a probabilidade de sobrevida global (SGLO). O teste de log rank e o modelo de regressão de Cox foram usados nas análises univariada e multivariada. Resultados: foram incluídos 159 pacientes, com mediana de tempo de seguimento de 13 meses. O intervalo entre o início dos sintomas e o diagnóstico teve mediana de 1,9 mês; 52% eram do gênero masculino. A mediana de idade ao diagnóstico foi de 7,2 anos. Os diagnósticos histológicos mais frequentes foram glioma de baixo grau (27%), meduloblastoma (19,5%) e tumor de tronco encefálico (17,6%). Cefaleia foi o sintoma mais frequente ao diagnóstico. A localização primária mais comum dos tumores foi a infratentorial (55,3%). A SGLO aos cinco anos foi de 42% (IC 95, 33 a 53%). Na análise univariada, foi observada associação significativa entre tempo de surgimento dos sintomas e o diagnóstico (p=0,046) e diagnóstico histológico (p<0,001). Na análise multivariada, esses fatores mantiveram-se e foram acrescidos da localização primária do tumor. Discussão: a SGLO observada neste estudo foi claramente inferior às descritas na literatura internacional, porém semelhante à de dois outros centros de referência nacionais. Estudos multicêntricos em nível nacional são necessários para confirmação desses resultados.

Objetives: To evaluate outcome of patients with the diagnosis of Central Nervous System tumors at a local reference pediatric oncology service, their clinical presentation, evolution and influence of prognostic factors. Methods: Patients enrolled in this retrospective study between March,2003 and December,2009, were less than nineteen years old at the time of the diagnosis. Kaplan-Meier method was used to estimate the overall survival (OS). Log rank test and Cox?s method were used in the statistical analysis. Results: Study population consisted of 159 patients with median follow up period of 13 months. Median of the time between onset of symptoms and diagnosis was 1,9 months. Boys comprised 52% of the studied population. Median age at diagnosis was 7,2 years. Most common diagnostic subtypes were low grade glioma (27%), medulloblastoma (19,5%) and brain stem tumors (17,6%). Headache was the most frequent symptom (57%) and infratentorial tumors correspond to 55,3% of the cases. An estimated OS of 42% (CI 95%, 33% to 53%) was observed for the role group. An association between outcome and the following variables was observed: interval between the time of onset of symptoms and diagnosis (p=0,046)and histological classification of the tumors (p<0,001). In the multivariate analysis, these variables were sustained, added of the primary location of the tumor in the Central Nervous System. Discussion: Overall survival detected in this study was clearly worse when compared with international centers rates, although were similar to other Brazilian studies. It?s important to develop multicentric studies to confirm data showed in this study

Mortalidad por cáncer infantil en Colombia durante 1985 a 2008 / Child mortality from cancer in Colombia, 1985-2008

OBJETIVO: Determinar la magnitud de la mortalidad por cáncer infantil en Colombia y evaluar las tendencias en su evolución entre 1985 y 2008. MÉTODOS: Se analizó durante dicho período la mortalidad en la población colombiana de 0 a 14 años provocada por cáncer en todas las localizaciones -leucemias, tumores malignos del sistema nervioso central (SNC), linfomas no Hodgkin, linfomas Hodgkin, tumores óseos y tumores renales. Se calculó el cambio promedio en las variaciones de las tendencias de mortalidad por cáncer en dicho grupo etario. RESULTADOS: Las muertes por cáncer constituyeron el 3,5 por ciento de la mortalidad en menores de 15 años. Entre los períodos 1985-1989 y 2005-2008 las tasas de mortalidad por cáncer mostraron un descenso en ambos sexos, pasando de 54,4 muertes por millón a 44,8 muertes por millón en niños y de 40,9 muertes por millón a 37,9 muertes por millón en niñas. La mortalidad por leucemias y linfomas registró un descenso estadísticamente significativo, mientras que la mortalidad por cánceres del SNC, contrariamente, aumentó también de manera significativa. CONCLUSIONES: Pese a leves tendencias a la baja en la mortalidad por leucemias y linfomas no Hodgkin, las tasas de mortalidad por cáncer infantil en Colombia permanecen altas y requieren esfuerzos importantes en los tratamientos para obtener mayores logros.

OBJECTIVE: Determine the magnitude of child mortality from cancer in Colombia and evaluate the trends in its evolution from 1985 to 2008. METHODS: Mortality in the Colombian population aged 0-14 years from cancer in any site (e.g., leukemia, malignant tumors of the central nervous system (CNS), nonHodgkin's lymphoma, Hodgkin's lymphoma, bone tumors, kidney tumors) during this period was analyzed. The mean change in the variations of cancer mortality trends in this age group was calculated. RESULTS: Deaths from cancer accounted for 3.5 percent of mortality in children under 15 years of age. During the periods 1985-1989 and 2005-2008 there was a decrease in mortality from cancer in both sexes, with figures dropping from 54.4 deaths per million to 44.8 deaths per million in boys and from 40.9 deaths per million to 37.9 deaths per million in girls. There was a statistically significant decrease in leukemia- and lymphoma-related mortality, whereas mortality associated with cancers of the CNS increased significantly. CONCLUSIONS: In spite of slight downward trends in mortality from leukemia and non-Hodgkin's lymphoma, childhood cancer mortality rates in Colombia remain high. Significant work on treatments for childhood cancer is required to obtain greater success.

CNS germ cell tumors: pattern of failure and effects of radiation volume.

This retrospective study was conducted to evaluate local control and overall survival after radiotherapy for patients with intracranial germ cell tumors and to investigate the influence of irradiated field on treatment outcome. Thirty-two patients with surgically confirmed or suspected primary intracranial germ cell tumors (GCT) treated at the Division of Therapeutic Radiology and Oncology, Chiang Mai University, Chiang Mai, Thailand between January 1988 and December 1999 were reviewed Seven patients were not included in the analysis of treatment outcome and survival due to incompleteness of radiation treatment or death before the end of treatment. The median follow up time of 39.5 months (range from 2.3 months to 136.1 months). Median age at diagnosis was 16.5 years with 23 males and 9 females. Patients were irradiated to the primary tumor with an adequate margin in 7 patients, to the whole brain with a cone down boost in 8 patients. Craniospinal irradiation (CSI) was performed in 10 patients. For the 25 evaluable patients, 5 year overall survival was 86.4%. Five-year disease free survival was 72.9%. Five year overall survival rates were 83.1% and 90.0% for the germinoma and nonbiopsied group. (p-value = 0. 6052). Routine prophylactic CSI was not given with a spinal only failure rate of 33.3%. Five-year overall survival were 85.7%, 87.5%, 85.7% for CSI, whole brain irradiation with boost and local field irradiation (p-value = 0.9037). Five-year disease free survival were 85.7%, 72.9%, 85.7% for CSI, WBRT, and local field (p-value = 0. 6403). This retrospective study suggests that definitive radiation therapy is effective in controlling germinoma, and cure rates are excellent with irradiation alone. Craniospinal irradiation can eliminate the risk of relapse especially in patients who had incomplete diagnostic craniospinal evaluation.

Survival improvement with combined radio-chemotherapy in the primary central nervous system lymphomas

The benefits of radio-chemotherapy in HIV-negative primary central nervous system (CNS) lymphomas were analyzed in 40 patients, who received radiotherapy to the brain or craniospinal axis with the total dose of 4460-5940 cGy to the primary tumor. Radiotherapy was followed by systemic chemotherapy, mainly with the cyclophosphamide, doxorubicin, vincristine and prednisolone (CHOP) regimen, in 16 of the patients. Follow-up ranged from four to 95 months with a median of 15 months. The relapse rate was 72.5%, and 83% of the relapses occurred within the radiation field. Median survival was 19 months and the two-year survival rate was 41%. Survival was significantly influenced by treatment method and radiation dose when measured by univariate analysis; median survival and the two-year survival rate was 29 months and 63% after radio-chemotherapy, while 13.5 month and 29% after radiotherapy alone (p= 0.027), and 22 months and 49% with doses of 50 Gy or more, but 12.5 months and 13% with doses less than 50 Gy (p=0.009). However, statistical significance was lost in multivariate analysis. These results might suggest the short-term efficacy of radio-chemotherapy, however, cautious observation is needed to confirm long-term effects.

Sarcoma meníngeo de alto grado de malignidad con invasión orbitaria: informe de un caso / Meningeal sarcoma of high degree of malignancy with orbitary invasion: a case report

Los sarcomas del sistema nervioso central son tumores primarios malignos, muy raros, que se originan de componentes de las meninges y que, por su baja frecuencia, sólo han sido consignado algunos casos de esta enfermedad en el Mundo. En el siguiente informe se presenta el caso de una paciente joven con un sarcoma meníngeo con invasión orbitaria. Se discuten aspectos clínicos, de neuroimagen y de histopatología, así como los factores pronósticos

Biópsia estereotáxica para linfomas primários do sistema nervoso central / Stereotaxic biopsy for primary lymphomas of the central nervous system

Os linfomas primários do SNC säo ratos, mas sua incidência tem aumentado nos últimos anos. Estes tumores frequentemente se localizaram nos gânglios de base e tálamo ou se apresentam de forma multifocal. O tratamento atual para eles é a radioterapia, associada ou näo a quimioterapia. Devido à sua extremamente baixa morbidade, a biópsia estereotáxica é o método ideal para determinar o diagnóstico histológico em pacientes com suspeita de linfomas do SNC. Os autoes apresentam estudo de 49 casos de linfomas primários do SNC, diagnosticado por biópsia esterotáxica