Roberto André Torres de Vasconcelos1,2, Pedro Guimarães Coscarelli3, Regina Papais Alvarenga2, Marcus André Acioly2,4,5. 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. 3. Universidade do Estado do Rio de Janeiro, Disciplina de Medicina Interna, 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.
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.
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.
Authors: Erin C Peckham-Gregory; Roberto E Montenegro; David A Stevenson; David H Viskochil; Michael E Scheurer; Philip J Lupo; Joshua D Schiffman Journal: J Neurooncol Date: 2018-04-16 Impact factor: 4.130
Authors: Roberto André Torres de Vasconcelos; Pedro Guimarães Coscarelli; Thyago Marsicano Vieira; Washington Silva Noguera; Davy Carlos Mendes Rapozo; Marcus André Acioly Journal: Cancer Med Date: 2019-02-08 Impact factor: 4.452
Authors: Sirkku Peltonen; Roope A Kallionpää; Matti Rantanen; Elina Uusitalo; Päivi M Lähteenmäki; Minna Pöyhönen; Janne Pitkäniemi; Juha Peltonen Journal: Int J Cancer Date: 2019-02-18 Impact factor: 7.396