悪性末梢神経鞘腫に対する外科的切除を受けた患者の臨床転帰
Melanie Alfonzo Horowitz1, Jawad M Khalifeh1, Xinlan Yang1
1Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
Background And Objectives:
Malignant peripheral nerve sheath tumors (MPNST) are aggressive soft tissue sarcomas with peripheral nerve differentiation. A wide surgical resection with negative margins is the mainstay of treatment but is not always curative. Here, we present clinical outcomes of patients who underwent surgical resection for MPNST.
Methods:
We identified and collected data on patients who underwent surgical resection for their MPNST at The Johns Hopkins Hospital, from 2010 to 2024. We generated Kaplan-Meier curves and performed univariable and multivariable analyses to determine factors associated with progression-free survival (PFS) and overall survival (OS).
Results:
We identified 123 MPNST patients. On univariable analysis, older age (hazard ratio [HR] 1.02), radiation-induced etiology (HR 1.59), spinal tumors (HR 3.27), high-grade pathology (HR 2.6), and postoperative complications (HR 3.07) were each associated with worse OS. Neurofibromatosis type 1 (NF1)-related etiology (HR 0.54), gross total resection (HR 0.48), negative margins (HR 0.58), R0 resection status (HR 0.46), and preoperative ambulatory status (HR 0.26) were each associated with improved OS. The results of the univariable analysis were similar for PFS and for OS and PFS within the NF1-related subgroup. On multivariable analysis, nonextremity MPNST (Spine adjusted hazard ratio [aHR] 3.28, Brachial Plexus aHR 5.51, Head/Neck aHR 6.23), recurrent tumor status (aHR: 2.64), and postoperative complications (aHR 3.27) were independently significantly associated with poor OS.
Conclusion:
MPNST are aggressive sarcomas that present challenges in diagnosis and treatment. In our series, NF1-related MPNST patients had the highest OS, likely associated with close monitoring for MPNST among the high-risk NF1-population. Nonextremity tumor locations, recurrent tumors, and postoperative complications were associated with inferior OS and PFS. Multi-institutional studies are warranted to investigate the impact of these prognostic factors in a larger, more heterogeneous MPNST patient cohort and examine the utility of surveillance in the neurofibromatosis patient population under a multidisciplinary team.
