Table 4.

Features that should heighten awareness about likelihood of MPNST

FeatureHow to assess
Large internal nodular neurofibroma burdenWhole-body MRI
Hard and painful subcutaneous neurofibroma(s)Clinical history and examination
Previously irradiated body regionClinical history
Germline NF1 microdeletion including SUZ12Mutation testing
Atypical neurofibroma (neurofibroma with regions of hypercellularity and nuclear atypia)Previous history and biopsy
Acute symptom/sign
Pain especially that wakes person at nightMRI and FDG PET
Focal neurologic sign/loss of functionMRI and FDG PET
Rapid growth of a neurofibroma or part of a plexiformMRI and FDG PET