An Assessment of Surgical Outcomes in Malignant Peripheral Nerve Sheath Tumors: A Systematic Review and Meta-Analysis of Surgical Interventions

Abdel-Hameed Al-Mistarehi, Department of Neurosurgery, Johns Hopkins School of Medicine, Baltimore, MD 21287, USA.
Khaled J. Zaitoun, Faculty of Medicine, Jordan University of Science and Technology, Irbid 22110, Jordan.
Jawad Khalifeh, Department of Neurosurgery, Johns Hopkins School of Medicine, Baltimore, MD 21287, USA.
Max A. Saint-Germain, Department of Neurosurgery, Johns Hopkins School of Medicine, Baltimore, MD 21287, USA.
Melanie Alfonzo Horowitz, Department of Neurosurgery, Johns Hopkins School of Medicine, Baltimore, MD 21287, USA.
Abdul Karim Ghaith, Department of Neurosurgery, Johns Hopkins School of Medicine, Baltimore, MD 21287, USA.
Chase H. Foster, Department of Neurosurgery, Johns Hopkins School of Medicine, Baltimore, MD 21287, USA.
Shoshana Braverman, Stern College for Women, Yeshiva University, New York, NY 10033, USA.
Avi N. Albert, School of Medicine, Meharry Medical College, Nashville, TN 37208, USA.
Usama AlDallal, School of Medicine, Medical University of Bahrain, Adliya P.O. Box 15503, Bahrain.
Allan Belzberg, Department of Neurosurgery, Johns Hopkins School of Medicine, Baltimore, MD 21287, USA.
Sang Lee, Department of Neurosurgery, Johns Hopkins School of Medicine, Baltimore, MD 21287, USA.
Nicholas Theodore, Department of Neurosurgery, Johns Hopkins School of Medicine, Baltimore, MD 21287, USA.
Ilya Laufer, Department of Neurosurgery, New York University Langone Health, New York, NY 10016, USA.
Daniel Lubelski, Department of Neurosurgery, Johns Hopkins School of Medicine, Baltimore, MD 21287, USA.

Document Type

Journal Article

Publication Date

6-15-2025

Journal

Cancers

Volume

17

Issue

12

DOI

10.3390/cancers17121997

Keywords

MPNST; PFS; malignant peripheral nerve sheath tumors; mortality; progression-free survival; recurrence; survival

Abstract

BACKGROUND/OBJECTIVES: Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive malignancies with a challenging prognosis, especially for patients with Neurofibromatosis type 1 (NF1). Their low incidence necessitates comprehensive studies to investigate the survival outcome. METHODS: We conducted a systematic review and meta-analysis, including data from 16 studies and 4265 patients, to explore surgical outcomes and survival rates, focusing on time-related outcomes, including overall survival (OS), progression-free survival (PFS), and recurrence rate. RESULTS: The analysis revealed that the OS rate was 86% [95% CI: 75-97%] at 1 year, decreasing to 60% [95% CI: 45-75%] at 3 years, and further declining to 47% [95% CI: 35-58%] by 5 years. For PFS, the 1-year rate was 61% [95% CI: 25-98%], which remained similar at 62% [95% CI: 35-89%] for 3 and 5 years. In NF1-associated MPNSTs, the 1-year OS was relatively high at 93% [95% CI: 83-100%], but it dropped to 68% [95% CI: 53-84%] at 3 years and further to 50% [95% CI: 31-68%] at 5 years. Additionally, the hazard ratio indicated a 38% lower survival rate in NF1 patients than those with sporadic MPNSTs when data were presented in the same study. Recurrence rates were high, with 56% of patients experiencing a relapse, primarily as local recurrences (70.6%). Mortality was significant, with over 50% of patients dying within an average follow-up period of 33.45 months. CONCLUSIONS: MPNSTs, particularly in NF1 patients, are associated with poor prognosis and high recurrence rates. These results underline the necessity of targeted therapeutic strategies and improved programs for screening, mainly through a multidisciplinary approach to optimize management.

Department

School of Medicine and Health Sciences Resident Works

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