Myxoid perineurioma: an entity with many mimics

We present a case of a female patient who presented with a 0.6cm flesh-colored "rubbery" papule on the left thigh. Biopsy revealed a dermal myxoid tumor containing spindled cells, tapered nuclei, indistinct cell borders, and a large number of mast cells. The spindle cells stained negative for S100 protein and Sox10 on immunohistochemistry, excluding myxoid neurofibroma, but positive for epithelial membrane antigen (EMA), and CD34, supporting a diagnosis of myxoid perineurioma. Interestingly, the mast cells showed cytoplasmic and nuclear positivity for microphthalmia transcription factor (MiTF). The lesion was fully excised one year later with identical histopathology and ancillary immunohistochemical profile.