Perineurial cell tumor (perineurioma) with granular cells

A form of benign cutaneous tumor with perineurioma findings and with the presence of associated granular cells is described. The two cases studied consisted of whorls made up of a high number of circumferentially arranged flattened cells, with perineurial characteristics, including bipolar cell processes, pinocytotic vesicles, a basal lamina, a positive immunoreactivity for EMA, and absence of immunostaining for S-100 protein. The granular cells, enclosed within the whorls, contained densely packed vesicles, particles with an apparently solid core, as well as membrane-limited vacuoles with disintegrating cellular organelles and electron-dense amorphous material. While failing to demonstrate any immunoreactivity for EMA, the granular cells showed positivity for S-100 protein, which supports their Schwann-cell origin. Due to its morphological and immunohistochemical characteristics, this peculiar form of tumor can be considered as a perineurioma with perineurial cell whorls and granular cell changes occurring in associated Schwann cells at the center of the whorls.