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

Gene rearrangement study for minimal residual disease monitoring in children with acute lymphocytic leukemia

OBJECTIVE To detect markers for minimal residual disease monitoring based on conventional polymerase chain reaction for immunoglobulin, T-cell receptor rearrangements and the Sil-Tal1 deletion in patients with acute lymphocytic leukemia. METHODS Fifty-nine children with acute lymphocytic leukemia from three institutions in Minas Gerais, Brazil, were prospectively studied. Clonal rearrangements were detected by polymerase chain reaction followed by homo/heteroduplex clonality analysis in DNA samples from diagnostic bone marrow. Follow-up samples were collected on Days 14 and 28-35 of the induction phase. The Kaplan-Meier and multivariate Cox methods were used for survival analysis. RESULTS Immunoglobulin/T-cell receptor rearrangements were not detected in 5/55 children screened (9.0%). For precursor-B acute lymphocytic leukemia, the most frequent rearrangement was IgH (72.7%), then TCRG (61.4%), and TCRD and IgK (47.7%); for T-acute lymphocytic leukemia, TCRG (80.0%), and TCRD and Sil-Tal deletion (20.0%) were the most common. Minimal residual disease was detected in 35% of the cases on Day 14 and in 22.5% on Day 28-35. Minimal residual disease on Day 28-35, T-acute lymphocytic leukemia, and leukocyte count above 50 x 109/L at diagnosis were bad prognostic factors for leukemia-free survival in univariate analysis. Relapse risk for minimal residual disease positive relative to minimal residual disease negative children was 8.5 times higher (95% confidence interval: 1.02-70.7). CONCLUSION Immunoglobulin/T-cell receptor rearrangement frequencies were similar to those reported before. Minimal residual disease is an independent prognostic factor for leukemia-free survival, even when based on a non-quantitative technique, but longer follow-ups are needed.

Apresentação clínica dos tumores sólidos mais comuns na infância e adolescência / Clinical presentation of the most common solid tumors in childhood and adolescent

O câncer infanto-juvenil é uma doença relativamente rara, sendo responsável por 0,5-3% do total de casos de câncer. Entretanto, ainda é causa de grande morbimortalidade em pacientes nessa faixa etária em nosso meio. Diagnóstico precoce e tratamento em centros especializados são essenciais para aumento da sobrevida em longo prazo. Objetivo: alertar o pediatra a respeito da importância do reconhecimento precoce das principais neoplasias malignas da infância e adolescência, dando ênfance à epedemiologia e apresentação clínica dos tumores sólidos. Métodos: foram selecionados 26 referências bibliográficas em pesquisa realizada nas base de dados Medline e no LILACS, em português, espanhol e inglês, utilizando-se as palavras-chave câncer infanto-juvenil, apresentação clínica, sobrevida.