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Published on: November 23, 2017
Outcomes Associated with Radiation Therapy for Localized Resectable Malignant Peripheral Nerve Sheath Tumors
Victoria Doss1, Elizabeth Sawdy1, Lindy Zhang2
1Department of Radiation Oncology and Molecular Radiation Sciences, Johns Hopkins University School of Medicine, Baltimore, Maryland.
Purpose:
Malignant peripheral nerve sheath tumor (MPNST) is a rare, aggressive soft tissue sarcoma with highest incidence in patients with neurofibromatosis type 1 (NF1). Data on outcomes and second malignancies following radiation therapy (RT) are limited, particularly in patients with NF1. This study evaluated overall survival (OS), progression-free survival (PFS), local failure, and secondary malignancies in patients with localized resectable MPNST.
Methods And Materials:
Patients diagnosed with localized resectable MPNST between 2010 and 2024 were retrospectively identified. Survival was estimated using the Kaplan-Meier method, multivariable analysis was conducted using Cox proportional hazards modeling, and cumulative incidence was estimated using Fine and Gray model.
Results:
Sixty-eight patients met inclusion criteria; median age was 34 years, and 75% had NF1. Median follow-up was 43.5 months. RT was delivered in 61.8% of patients, primarily preoperatively (71.1%). RT was associated with improved 3-year OS (87.2% vs 51.5%, P = .039) and 3-year PFS (55.8% vs 41.5%, P = .017). On multivariable analysis, RT remained independently associated with improved PFS (HR, 0.34, P = .003) and OS (HR, 0.4, P = .034). Three-year local failure was for RT versus no RT was 26.3% versus 35.3% (P = .240). One secondary malignancy arose within a prior RT field 9.5 years after initial RT, and another at the RT field edge 8.6 years later.
Conclusions:
RT was independently associated with improved PFS and OS in patients with localized resected MPNST. These hypothesis-generating findings need validation in a larger cohort. The risk of RT-associated secondary malignancy was low, occurring beyond the median disease progression timeframe.
