MPNST without muscle weakness at presentation: an analysis of an underappreciated combination

Malignant peripheral nerve sheath tumors (MPNSTs) of major motor nerves typically present with muscle weakness and pain. We aim to analyze and characterize patients with MPNST at major motor nerves but without muscle weakness at initial presentation. A retrospective search involving MPNSTs in a major nerve evaluated and/or treated at our institution from 1994 to 2019 was performed. Patients with no muscle weakness and available MRI were analyzed. Clinical materials, MR images and PET scans were reviewed for features of malignancy. This group of patients was compared to patients who presented with MPNSTs and muscle weakness. Twenty-six patients were included in the no muscle weakness group. Of them, twenty-one (81%) patients had a positive family history for malignancy. Only 16 (62%) MR images were highly suspicious for malignancy. All 7 available PET-scans were highly suspicious for malignancy. Patients who presented with muscle weakness (n = 36), were more likely to have paresthesias and a history of NF-1 or radiation to the MPNST location (p-value < 0.05). MPNSTs of major motor nerves without muscle weakness represent an underappreciated subset of cases which has potential treatment and outcome implications. These patients presented with fewer symptoms and had fewer risk factors than patients with muscle weakness. PET-scans should be considered as an extra method of trying to anticipate the diagnosis of an MPNST.