We present here four cases of extraneural perineurioma (EP), which occurred in 31, 32, 42 and 43 year old women in the left hand, right arm (2 cases) and right axillary regions, respectively. All tumors were well circumscribed, with a thin capsule. They were 2, 2, 1.5 and 1.5 cm in the largest diameter. Microscopically all tumors had a distinctive appearance composed of interconnected cords of cells which ran in a parallel fashion or formed a characteristically looking cellular lattice. These cords were composed of cells with tapered to stellate shaped nuclei having one or two inconspicuous nucleoli. The space between the cells of the lattice was edematous and empty looking, or it was composed of myxoid matrix, or it was filled with characteristically looking globular deposits of collagen extracellular matrix. These globular deposits of collagen extracellular matrix often indented the nuclei, giving them a stellar appearance. Immunohistochemically all tumors reacted positively with antibodies to epithelial membrane antigen. antibodies to smooth muscle actin, muscle-specific actin, S-100 protein, cytokeratin AE1/AE3, cytokeratin CAM 5.2, and HMB-45 gave uniformly negative immunohistochemical results.
