Malignant peripheral nerve sheath tumor with and without neurofibromatosis type 1 / Tumor maligno da bainha de nervo periférico com e sem Neurofibromatose tipo 1

ABSTRACT Objective In this study, we review the institution’s experience in treating malignant peripheral nerve sheath tumors (MPNSTs). A secondary aim was to compare outcomes between MPNSTs with and without neurofibromatosis type 1 (NF1). Methods Ninety-two patients with MPNSTs, over a period of 20 years, were reviewed. A retrospective chart review was performed. The median age was 43.5 years (range, 3–84 years) and 55.4% were female; 41 patients (44.6%) had NF1-associated tumors. Results Mean tumor sizes were 15.8 ± 8.2 cm and 10.8 ± 6.3 cm for patients with and without NF1, respectively. Combined two- and five-year overall survival was 48.5% and 29%. Multivariate analysis confirmed the association of tumor size greater than 10 cm (hazard ratio (HR) 2.99; 95% confidence interval (CI) 1.14–7.85; p = 0.0258) and presence of NF1 (HR 3.41; 95%CI 1.88–6.19; p < 0.001) with a decreased overall survival. Conclusion Tumor size and NF1 status were the most important predictors of overall survival in our population.

RESUMO Objetivo Relatamos a experiência institucional no tratamento de tumores malignos da bainha de nervo periférico (TMBNP) e comparamos o prognóstico entre pacientes com e sem neurofibromatose tipo 1 (NF1). Métodos Foram incluídos neste estudo 92 pacientes num período de 20 anos. Foi realizada uma análise retrospectiva dos prontuários, das características do tumor e do tratamento. A idade mediana era 43,5 anos (variação 3–84 anos) e 55,4% dos pacientes eram mulheres; 41 pacientes (44,6%) tinham tumores associados à NF1. Resultados O diâmetro médio dos tumores era 15,8 ± 8,2cm e 10,8 ± 6,3cm para pacientes com e sem NF1, respectivamente. A sobrevida combinada em 2 e 5 anos foi de 48,5% e 29%. A análise multivariada confirmou que o tamanho do tumor acima de 10cm (hazard ratio (HR) 2.99; 95% intervalo de confiança (IC) 1.14–7.85; p = 0.0258) e a presença de NF1 (HR 3.41; 95%IC 1.88–6.19; p < 0.001) estão associados a uma pior sobrevida. Conclusões O tamanho do tumor e a associação com NF1 foram os preditores mais importantes de sobrevida na nossa população.

A relação entre o nível de multimorbidade e o de qualidade de vida relacionada à saúde: influência da personalidade e do suporte social / The relationship of multimorbidity with health-related quality of life: the influence of personality and social support

Justificativa: A influência da personalidade e do suporte social na qualidade de vida relacionada à saúde (HRQoL) foi previamente demonstrada em estudos cujas amostras são compostas por pacientes portadores de um único diagnóstico, dificultando a generalização para portadores de multimorbidade. Para reduzir esta lacuna, foi desenvolvido este estudo sobre a influência da personalidade e do suporte social na relação entre multimorbidade e HRQoL em um grupo de pacientes com alta prevalência de multimorbidade. Desenho Experimental: O estudo de corte transversal incluiu 178 pacientes aacompanhados em um ambulatório do Hospital Universitário Pedro Ernesto (HUPE). Em geral, esses indivíduos são portadores de múltiplas patologias comórbidas, muitas delas incomuns e de apresentação atípica. A HRQoL foi avaliada através do questionário SF-36, a multimorbidade através de ma medida de carga total de doença: a Cummulative Illness Rating Scale (CIRS), a personalidade pelo Cloninger's Temperament and Character Inventory (TCI) e o suporte social pelo Sarason's Social Support Questionnaire (SSQ). Também foram incluídos no estudo para a avaliação de psicopatologia o Symptoms Checklist-90 (SCL-90) e o inventário de depressão de Beck. A associação entre multimorbidade e HRQoL, a influência da personalidade e do suporte social, e o ajuste pela psicopatologia foram analisados pela elaboração de modelos lineares generalizados. Resultados: A multimorbidade se associa com cinco das oito subescalas mensuradas pelo SF-36: capacidade funcional (CF), estado geral de saúde (EGS), vitalidade (VIT), aspectos sociais (AS) e saúde mental (SM). Após a introdução nos modelos das dimensões de personalidade, o efeito da multimorbidade desaparece nas subescalas CF e SM, sugerindo mediação, mas mantém-se nas subescalas EGS, VIT e AS. As dimensões de personalidade harm avoidance e self-directedness são aquelas com maior influência nos modelos. Limitações do estudo não permitiram que analisássemos o efeito do suporte social (pequeno número de respondentes), nem fizéssemos o ajuste dos modelos pela psicopatologia (artefatos estatísticos provavelmente por grande colinearidade das variáveis). Conclusões: Em indivíduos com alta prevalência de multimorbidade, esta se associa a diversas dimensões de HRQoL e algumas dimensões de personalidade influenciam nessa associação.

Background: Previous research has shown the influence of personality and social support on health-related quality of life (HRQoL) in patients with single diagnosis, limiting generalization of the results to patientes with multimorbidity. We conduct a study of the influence of personality and social support on health-related quality of life in a group of patients with high prevalence of multimorbidity. Experimental Design: This is a cross-sectional study of 178 subjects seen in a ambulatory setting in Hospital Universitário Pedro Ernesto, Rio de Janeiro (RJ), Brazil. These patients are expected to have multiple comorbid diseases, including rare and atypical presentations. The HRQoL was measured with the SF-36; multimorbidity with the Cummulative Illness Rating Scale (CIRS) - a measure of burden of disease; personality with the Cloninger's Temperament and Character Inventory (TCI); and social support with the Sarason's Social Support Questionnaire (SSQ). Psychopathology was evaluated with the Symptoms Checklist-90 (SCL-90) and the Beck Depression Inventory. Regression analysis with generalized linear models was used examine the association of multimorbidity and HRQoL and the influence of personality and social support. Psycopathology was intended to be included as covariates in the models. Results: Multimorbidity is associated with five of eight HRQoL SF-36 subscales: physical functioning (PF), general health (GH), vitality (VI), role social (RS) e mental health (MH). After the inclusion of the personality dimensions in the models, the effect of multimorbidity vanishes in the subscales PF and MH, suggesting a mediation effect, but not in the subscales GH, VI, RS. The personality dimensions harm avoidance and self-directedness have the largest effects on HRQoL. The limitations of the study includes the small number of respondents of the social support questionnaire and the presence of statistical artifacts - probably due to colinearity - that precludes the inclusion of psychopathology in the models. Conclusions: In a group of subjects with an expected high prevalence of multimorbidity, the burden of disease is associated with most dimensions of HRQoL. We also observed some personality dimensions influences this association.

Lamotrigine as an adjuvant treatment for acute bipolar depression: a Brazilian naturalistic study

Lamotrigine is indicated according to several recent treatment guidelines as a first-line medication for the treatment of bipolar depression. However, its efficacy in acute bipolar depression has not been well established. In the present naturalistic study, patients with bipolar depression (n = 20), predominantly bipolar type I, were treated with lamotrigine in addition to their prior treatment for 8 weeks. The Young Mania Rating Scale (YMRS), 17-item Hamilton Rating Scale for Depression (HAM-D-17), and Clinical Global Impressions-Bipolar Disorder (CGI-BD) scale were applied at baseline, week 4, and week 8. With regard to the primary measure of efficacy, mean total HAM-D-17 scores significantly decreased (p < .01) at the end of treatment. Eight patients (40%) exhibited a positive response (i.e., at least a 50% reduction of baseline scores). Additionally, eight (40%) and 11 (55%) patients exhibited complete remission, reflected by HAM-D-17 and CGI-BP scores, respectively. Episodes of switching to mania or hypomania occurred in five patients (25%). No skin rash or any other significant adverse events were reported. Our results indicate that the addition of lamotrigine to a mood stabilizer can be useful in the treatment of acute depressive episodes in bipolar I disorder.