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Updated: Feb 27, 2026
Author Spotlight: Genetically Engineered Mouse Models and Pathological Characterization of Neurofibromatosis Type 1 Associated Tumors
Published on: May 17, 2024
Malignant peripheral nerve sheath tumor with and without neurofibromatosis type 1
Roberto André Torres de Vasconcelos1,2, Pedro Guimarães Coscarelli3, Regina Papais Alvarenga2
1Instituto Nacional do Câncer, Departamento de Oncologia Cirúrgica, Divisão de Osso e Tecido Conjuntivo, Rio de Janeiro RJ, Brasil.
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.
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