Malignant peripheral nerve sheath tumor with and without neurofibromatosis type 1

被引：1
|
作者：
Torres de Vasconcelos, Roberto Andre [1 ,2 ]
Coscarelli, Pedro Guimaraes [3 ]
Alvarenga, Regina Papais [2 ]
Acioly, Marcus Andre [2 ,4 ,5 ]
机构：
[1] Inst Nacl Canc, Dept Oncol Cirurg, Div Osso & Tecido Conjunt, Rio De Janeiro, RJ, Brazil
[2] Univ Fed Estado Rio de Janeiro, Programa Posgrad Neurol, Rio De Janeiro, RJ, Brazil
[3] Univ Estado Rio de Janeiro, Disciplina Med Interna, Rio De Janeiro, RJ, Brazil
[4] Univ Fed Rio de Janeiro, Disciplina Neurocirurgia, Rio De Janeiro, RJ, Brazil
[5] Univ Fed Fluminense, Disciplina Neurocirurgia, Niteroi, RJ, Brazil
来源：
ARQUIVOS DE NEURO-PSIQUIATRIA | 2017年 / 75卷 / 06期
关键词：
nerve sheath tumors; neurofibromatosis; 1; survival; QUALITY-OF-LIFE; SOFT-TISSUE SARCOMAS; PROGNOSTIC-FACTORS; SURVIVAL; OUTCOMES;
D O I：
10.1590/0004-282X20170052
中图分类号：
Q189 [神经科学];
学科分类号：
071006 ;
摘要：
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.
引用
收藏
页码：366 / 371
页数：6
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