The role of radiotherapy in MPNST and the impact of NF1 status on outcomes: Insights from a multicenter cohort study.

ABSTRACT:

Background

Malignant peripheral nerve sheath tumors (MPNSTs) are rare, aggressive sarcomas, with 40% associated with neurofibromatosis type 1 (NF1). Surgical excision is the main treatment for localized disease; however, local recurrence (LR) remains high. Radiotherapy (RTx) is increasingly used to enhance local control in STS, but its use remains controversial due to the potential for increased major wound complications and an increased risk of secondary malignancies in NF1 patients. This study investigated the use and impact of RTx on local control in MPNSTs, particularly in the NF1 setting.

Methods

Surgically treated primary MPNSTs from 1988 to 2019 in the MONACO multicenter cohort were included. Differences in demographics, treatment, and RTx use between NF1 and non-NF1

SUBMITTER: Jansma CYMN 

PROVIDER: S-EPMC12916730 | biostudies-literature | 2025 Dec

REPOSITORIES: biostudies-literature