Malignant peripheral nerve sheath tumor with and without neurofibromatosis type 1

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.

Object Details

• Alternative title: Tumor maligno da bainha de nervo periférico com e sem Neurofibromatose tipo 1
• Location: Deutsche Nationalbibliothek Frankfurt am Main
• Extent: Online-Ressource
• Language: Englisch

References and Relationships

• Bibliographic citation: Malignant peripheral nerve sheath tumor with and without neurofibromatosis type 1 ; volume:75 ; number:06 ; year:2017 ; pages:366-371 ; Arquivos de neuro-psiquiatria ; 75, Heft 06 (2017), 366-371

Contributors, Places and Time

• Contributor: Vasconcelos, Roberto André Torres de ; Coscarelli, Pedro Guimarães ; Alvarenga, Regina Papais ; Acioly, Marcus André

Further information

• DOI: 10.1590/0004-282X20170052
• URN: urn:nbn:de:101:1-2023101911270590249657
• Rights: Open Access; Der Zugriff auf das Objekt ist unbeschränkt möglich.
• Last update: 14.08.2025, 10:52 AM CEST