Malignant peripheral nerve sheath tumor with and without neurofibromatosis type 1

Vasconcelos, Roberto Andre Torres de; Coscarelli, Pedro Guimarães; Alvarenga, Regina Papais; Acioly, Marcus André

Please use this identifier to cite or link to this item: https://ninho.inca.gov.br/jspui/handle/123456789/5324

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DC Field	Value	Language
dc.contributor.author	Vasconcelos, Roberto Andre Torres de	-
dc.contributor.author	Coscarelli, Pedro Guimarães	-
dc.contributor.author	Alvarenga, Regina Papais	-
dc.contributor.author	Acioly, Marcus André	-
dc.date.accessioned	2022-02-25T14:10:15Z	-
dc.date.available	2022-02-25T14:10:15Z	-
dc.date.issued	2017	-
dc.identifier.citation	VASCONCELOS, Roberto Andre Torres de et al. Malignant peripheral nerve sheath tumor with and without neurofibromatosis type 1. Arq Neuropsiquiatr, v. 75, n. 6, p. 366-371, 2017.	-
dc.identifier.issn	1678-4227	-
dc.identifier.uri	http://sr-vmlxaph03:8080/jspui/handle/123456789/5324	-
dc.description	p. 366-371.: il. p&b.	-
dc.description.abstract	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.	-
dc.publisher	Arq Neuropsiquiatr	pt_BR
dc.subject	Neoplasias da Bainha Neural	pt_BR
dc.subject	Neurofibromina 1	pt_BR
dc.subject	Neurofibromin 1	pt_BR
dc.subject	Sobrevida	pt_BR
dc.subject	Survival	pt_BR
dc.subject	Nerve Sheath Neoplasms	pt_BR
dc.title	Malignant peripheral nerve sheath tumor with and without neurofibromatosis type 1	pt_BR
dc.title.alternative	Tumor maligno da bainha de nervo periférico com e sem Neurofibromatose tipo 1	pt_BR
dc.Type	Article	pt_BR
dc.description.abstracten	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.	-
Appears in Collections:	Artigos de Periódicos da área de Tecido Ósseo e Conectivo

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