Malignant peripheral nerve sheath tumors in neurofibromatosis type 1 arise from distinct nodular lesions: A retrospective imaging analysis.

ABSTRACT:

Background

People with neurofibromatosis type 1 (NF1) have an 8%-13% lifetime risk of developing malignant peripheral nerve sheath tumors (MPNST). Atypical neurofibromas (AN) and atypical neurofibromatous neoplasms of uncertain biologic potential (ANNUBP) have been described as precursors to MPNST. AN/ANNUBP often appear as distinct nodular lesions (DNL) that are ≥3 cm in longest diameter. To assess the hypothesis that most MPNST originate in preexisting DNL, we performed a retrospective review of MRIs obtained prior to the MPNST diagnosis in people with NF1.

Methods

Study subjects were identified at two NF centers, University Hospital Hamburg-Eppendorf in Germany and the National Cancer Institute in Bethesda, Maryland, USA. We confirmed the location of MPNST on the MRI perf

SUBMITTER: Salamon J 

PROVIDER: S-EPMC12746603 | biostudies-literature | 2025 Jan-Dec

REPOSITORIES: biostudies-literature