Who cares for adolescents and young adults with cancer in Brazil? / Onde são tratados os adolescentes e jovens adultos com câncer no Brasil?

Abstract Objective: Approximately 6% of all cancers arise in adolescents and young adults. Currently, the ward type best placed to treat this patient group remains controversial. The aim of this study was to evaluate exactly where adolescents and young adults with cancer are treated in Brazil. Methods: Data were extracted from 271 Brazilian hospital-based cancer registries (2007-2011), including all five national regions (North, Northeast, Midwest, South, and Southeast). Variables included gender, age, ethnicity, National Code of Health Establishment, hospital unit state, and region. Tumors were classified according to the World Health Organization classification for adolescents and young adults with cancer. Odds ratios with 95% confidence intervals were computed by unconditional logistic regression. Results: Most patients were managed on medical oncology wards, followed by pediatric oncology and then by non-specialist wards. Of patients aged 15-19 years, 49% were managed on pediatric wards; most of the older patients (96%; aged 20-24) were managed on adult wards. Patients were more likely to be seen in medical oncology wards as their age increased (OR = 2.03 [1.98-2.09]), or if they were based in the South (OR = 1.50 [1.29-1.73]). Conversely, bone tumors were less likely to be treated (decreased OR) on medical oncology wards, regardless of age, gender, and region. Conclusion: An elevated risk of treatment on medical oncology wards was observed for older patients and those treated in the South. Bone tumors were generally treated in pediatric oncology wards, while skin cancers were treated in medical oncology wards, regardless of age, gender, and region.

Resumo Objetivo: Aproximadamente 6% de todos os cânceres surgem em adolescentes e adultos jovens. Atualmente, o melhor tipo de enfermaria para tratar esse grupo de pacientes continua sendo controverso. O objetivo deste estudo foi avaliar exatamente onde os adolescentes e adultos jovens com câncer são tratados no Brasil. Métodos: Foram coletados dados de 271 registros de câncer de base hospitalar (2007-2011), inclusive de todas as cinco regiões nacionais (Norte, Nordeste, Centro-Oeste, Sul e Sudeste). As variáveis incluíram sexo, idade, etnia, o Código Nacional de Estabelecimento de Saúde e o estado e a região da unidade hospitalar. Os tumores foram classificados de acordo com a classificação da Organização Mundial de Saúde para adolescentes e adultos jovens com câncer. As razões de chance com intervalos de confiança de 95% foram calculadas por regressão logística incondicional. Resultados: A maioria dos pacientes foi tratada em enfermaria de oncologia médica, seguido da enfermaria de oncologia pediátrica e, então, a enfermaria sem especialidade. 49% dos pacientes entre 15-19 anos foram tratados em enfermarias pediátricas; os pacientes mais velhos (96%, entre 20-24) foram tratados em enfermarias de adultos. Os pacientes apresentaram maior propensão a serem vistos em enfermarias de oncologia conforme mais velhos (RC = 2,03 [1,98-2,09]) ou caso morassem na região Sul (RC = 1,50 [1,29-1,73]). Por outro lado, os tumores ósseos mostraram menor propensão a tratamento (redução da RC) em enfermarias de oncologia, independentemente da idade, sexo e região. Conclusão: Foi visto um risco elevado de tratamento, em enfermarias de oncologia, de pacientes mais velhos e os tratados na Região Sul. Os tumores ósseos foram, em geral, tratados em enfermarias de oncologia pediátrica, ao passo que os cânceres de pele foram tratados em enfermarias de oncologia médica, independentemente de idade, sexo e região.

Birth weight and risk of childhood solid tumors in Brazil: a record linkage between population-based data sets / El peso al nacer y el riesgo de formación de tumores sólidos en la niñez en el Brasil: vinculación de registros entre conjuntos de datos poblacionales

ABSTRACT Objective To analyze the relationship between the development of childhood solid tumors and 1) birth weight and 2) fetal growth, using two Brazilian population-based data sets. Methods A case–cohort study was performed using two population-based data sets, and linkage between the Live Birth Information System (Sistema de Informação sobre Nascidos Vivos, SINASC) and 14 population-based cancer registries (PBCRs) was established. Four controls per case were chosen randomly from the SINASC data set. Tumors were classified as central nervous system (CNS), non-CNS embryonal, and other tumors (“miscellaneous”). Adjustments were made for potential confounders (maternal age, mode of delivery, maternal education, birth order, gestational age, sex, and geographic region). Odds ratios (ORs) with 95% confidence intervals (CIs) were computed using unconditional logistic regression analysis. Results In a trend analysis, for every 500 g of additional birth weight, the crude OR was 1.12 (CI: 1.00–1.24) and the adjusted OR was 1.02 (CI: 0.90–1.16) for all tumors. For every 1 000 g of additional birth weight, the crude OR was 1.25 (CI: 1.00–1.55) and the adjusted OR was 1.04 (CI: 0.82–1.34) for all tumors. Among children diagnosed after reaching the age of 3 years, in the miscellaneous tumor category, the OR was significantly increased for every additional 500 g and 1 000 g of birth weight. Conclusions The study data suggested that increased birth weight was associated with childhood solid tumor development, especially among children more than 3 years old with “miscellaneous” tumors.

RESUMEN Objetivo Analizar la relación entre la aparición de tumores sólidos en la niñez y 1) el peso al nacer y 2) el crecimiento fetal, a partir de dos conjuntos de datos poblacionales del Brasil. Métodos Se efectuó un estudio de casos en una cohorte a partir de dos conjuntos de datos poblacionales y se vinculó el sistema de información de nacidos vivos (Sistema de Informação sobre Nascidos Vivos, SINASC) con 14 registros oncológicos poblacionales. Se eligieron al azar cuatro controles por caso del conjunto de datos del SINASC. Los tumores se clasificaron en tres tipos: del sistema nervioso central (SNC), embrionarios ajenos al SNC y otros (“misceláneos”). Se hicieron ajustes en función de los posibles factores de confusión (edad materna, modalidad de parto, educación materna, orden de nacimiento, edad gestacional, sexo y región geográfica) y se calcularon las razones de posibilidad (OR) con un intervalo de confianza (IC) del 95 % mediante análisis de la regresión logística incondicional. Resultados En el análisis de las tendencias, se observó que, en todos los tumores, cada 500 g adicionales de peso al nacer la OR bruta fue de 1,12 (IC: 1,00-1,24) y la OR ajustada, de 1,02 (IC: 0,90-1,16), mientras que, cada 1 000 g adicionales, la OR bruta fue de 1,25 (IC: 1,00-1,55) y la OR ajustada, de 1,04 (IC: 0,82-1,34). En cuanto a los niños diagnosticados después de los 3 años de edad, en la categoría de tumores misceláneos, la OR fue significativamente más alta con cada 500 g y 1 000 g adicionales de peso al nacer. Conclusiones Los datos del estudio indican que el peso alto al nacer está asociado a la aparición de tumores sólidos en la niñez, especialmente de la categoría “misceláneos” y en los niños mayores de 3 años de edad.

A de novo em ~1.3 Mb microdeletion at 17q11.2 associated with Neurofibromatosis-Noonan syndrome

Introduction: Neurofibromatosis-Noonan syndrome is a clinical entity considered an extended Neurofibromatosis phenotype generally caused by different types of intragenic mutations at the NF1 gene. About 5%-10% of patients with neurofibromatosis diagnosis carry chromosomal microdeletions involving NF1, often presenting with a more severe phenotype than that observedin the patients carrying intragenic mutations; however, anticipating the presence of a deletion based only in the phenotype is not straightforward. Patient and Methods: Here we investigated by oligoarray-CGH (aCGH) the presence of a submicroscopic genomic rearrangement in a patientwith a clinical picture of Neurofibromatosis, and other characteristics compatible with Noonansyndrome. Results: The aCGH analysis revealed a germline de novo ~1.3 Mb microdeletion at 17q11.2 encompassing other coding genes besides the NF1 gene. Discussion: Up to now, thenumber of reported patients with Neurofibromatosis-Noonan syndrome carrying NF1 microdeletions is quite small. The continuous identification of patients carrying 17q11.2 deletions canhelp to establish a reliable genotype-phenotype relationship in this syndrome