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CC BY-NC-ND 4.0 · Arq Neuropsiquiatr 2017; 75(06): 366-371
DOI: 10.1590/0004-282X20170052
ARTICLE

Malignant peripheral nerve sheath tumor with and without neurofibromatosis type 1

Tumor maligno da bainha de nervo periférico com e sem Neurofibromatose tipo 1
Roberto André Torres de Vasconcelos
1   Instituto Nacional do Câncer, Departamento de Oncologia Cirúrgica, Divisão de Osso e Tecido Conjuntivo, Rio de Janeiro RJ, Brasil;
2   Universidade Federal do Estado do Rio de Janeiro, Programa de Pós-Graduação em Neurologia, Rio de Janeiro RJ, Brasil;
,
Pedro Guimarães Coscarelli
3   Universidade do Estado do Rio de Janeiro, Disciplina de Medicina Interna, Rio de Janeiro RJ, Brasil;
,
Regina Papais Alvarenga
2   Universidade Federal do Estado do Rio de Janeiro, Programa de Pós-Graduação em Neurologia, Rio de Janeiro RJ, Brasil;
,
Marcus André Acioly
2   Universidade Federal do Estado do Rio de Janeiro, Programa de Pós-Graduação em Neurologia, Rio de Janeiro RJ, Brasil;
4   Universidade Federal do Rio de Janeiro, Disciplina de Neurocirurgia, Rio de Janeiro RJ, Brasil;
5   Universidade Federal Fluminense, Disciplina de Neurocirurgia, Niterói RJ, Brasil.
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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.

nerve sheath tumors - neurofibromatosis 1 - survival
neoplasias de bainha neural - neurofibromatose tipo 1 - sobrevivência


Publication History

Received: 28 September 2016

Accepted: 14 February 2017

Article published online:
05 September 2023

© 2023. Academia Brasileira de Neurologia. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commecial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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