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An Assessment of Surgical Outcomes in Malignant Peripheral Nerve Sheath Tumors: A Systematic Review and Meta-Analysis of Surgical Interventions
by
, , , , ,
Abdel-Hameed Al-Mistarehi
1
,
Khaled J. Zaitoun
2,
Jawad Khalifeh
1,
Max A. Saint-Germain
1,
Melanie Alfonzo Horowitz
1,
Abdul Karim Ghaith
1,
Chase H. Foster
1,3
,
Shoshana Braverman
4,
Avi N. Albert
5,
Usama AlDallal
6,
Allan Belzberg
1,
Sang Lee
1,
Nicholas Theodore
1,
Ilya Laufer
7 and
Daniel Lubelski
1,* 1
Department of Neurosurgery, Johns Hopkins School of Medicine, Baltimore, MD 21287, USA
2
Faculty of Medicine, Jordan University of Science and Technology, Irbid 22110, Jordan
3
Department of Neurosurgery, School of Medicine, George Washington University, Washington, DC 20037, USA
4
Stern College for Women, Yeshiva University, New York, NY 10033, USA
5
School of Medicine, Meharry Medical College, Nashville, TN 37208, USA
6
School of Medicine, Medical University of Bahrain, Adliya P.O. Box 15503, Kingdom of Bahrain
7
Department of Neurosurgery, New York University Langone Health, New York, NY 10016, USA
*
Author to whom correspondence should be addressed.
Cancers 2025, 17(12), 1997; https://doi.org/10.3390/cancers17121997
Submission received: 7 April 2025
/
Revised: 3 June 2025
/
Accepted: 12 June 2025
/
Published: 15 June 2025
(This article belongs to the Special Issue Advances in Spine Oncology: Research and Clinical Studies)
Simple Summary
Malignant peripheral nerve sheath tumors (MPNSTs) are rare soft tissue sarcomas with peripheral nerve sheath differentiation, which tend to be locally aggressive, with systemic metastasis and poor survival outcomes. Although we understand MPNST pathophysiology, less is known about the time-to-event metrics, such as mortality, progression-free survival, and overall survival. In this study, we sought to characterize these time-to-event metrics and survival outcomes by conducting a thorough systematic review of published studies of surgically managed MPNST. Understanding these metrics will emphasize the need for targeted therapeutic strategies and improved screening programs for patients with MPNST and encourage multidisciplinary collaboration to optimize management of these tumors.
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.
