Imaging of non-neurogenic peripheral nerve malignancy-a case series and systematic review

To evaluate the frequency, clinico-pathologic and imaging features of malignant tumors in peripheral nerves which are of non-neurogenic origin (non-neurogenic peripheral nerve malignancy-PNM).

We retrospectively reviewed our pathology database for malignant peripheral nerve tumors from 07/2014-07/2019 and performed a systematic review. Exclusion criteria were malignant peripheral nerve sheath tumor (MPNST). Clinico-pathologic and imaging features, apparent diffusion coefficient (ADC_min), and standard uptake values (SUV_max) are reported.

After exclusion of all neurogenic tumors (benign = 196, MPNST = 57), our search yielded 19 non-neurogenic PNMs (7%, n = 19/272), due to primary intraneural malignancy (16%, n = 3/19) and secondary perineural invasion from an adjacent malignancy (16%, n = 3/19) or metastatic disease (63%, n = 12/19). Non-neurogenic PNMs were located in the lumbosacral plexus/sciatic nerves (47%, n = 9/19), brachial plexus (32%, n = 6/19), femoral nerve (5%, n = 1/19), tibial nerve (5%, n = 1/19), ulnar nerve (5%, n = 1/19), and radial nerve (5%, n = 1/19). On MRI (n = 14/19), non-neurogenic PNM tended to be small (< 5 cm, n = 10/14), isointense to muscle on T1-W (n = 14/14), hyperintense on T2-WI (n = 12/14), with enhancement (n = 12/12), low ADC_min (0.5-0.7 × 10-3 mm²/s), and variable metabolic activity (SUV_max range 2.1-13.1). A target sign was absent (n = 14/14) and fascicular sign was rarely present (n = 3/14). Systematic review revealed 89 cases of non-neurogenic PNM.

Non-neurogenic PNMs account for 7% of PNT in our series and occur due to metastases and primary intraneural malignancy. Although non-neurogenic PNMs exhibit a non-specific MRI appearance, they lack typical signs of neurogenic tumors such as the target sign. Quantitative imaging features identified by DWI (low ADC) and F¹⁸-FDG PET/CT (high SUV) may be helpful clues to the diagnosis.