Review Article

Malignant Peripheral Nerve Sheath Tumors State of the Science: Leveraging Clinical and Biological Insights into Effective Therapies

Figure 1

Pathogenesis of peripheral nerve sheath tumors in NF1. Percentages below each tumor type is the range of lifetime prevalence in individuals with NF1. Representative clinical photograph (a), MRI imaging (b), histology (c), clinical symptomology (d), and genetic features (e) of each tumor type are given. Histologically, plexiform neurofibroma shows mixture of areas of hypercellularity in the absence of other atypical features. Atypical neurofibroma shows atypical nuclei and higher cellularity. In contrast, MPNST are highly cellular with high mitotic activity and areas of necrosis.