Sclerosing perineurioma: A tumor variant?

We describe two examples of soft tissue perineurioma, a hard tumor, 1.7 cm in diameter, involving the dermis and superficial subcutaneous tissue of the left thumb, and the other a massive, lobulated lesion of the pelvis. Both occurred in 49-year-old men. Both were mobile and well demarcated but unencapsulated. Their cut surfaces were white and collagenous. Microscopically, each consisted of spindle-shaped or epithelioid tumor cells in a dense stroma of haphazardly oriented collagen bundles. Spindle-shaped cells were individually distributed, whereas epithelioid cells were often arranged in cords or clusters. Cytologic atypia was conspicuous in the massive tumor, but no mitotic activity was observed. Immunostains showed diffuse reactivity for vimentin, membrane pattern staining for epithelial membrane antigen and type IV collagen, as well as patchy CD34 reactivity. Stains for S-100 protein, Leu-7, smooth muscle actin, desmin, and cytokeratin were negative. Ultrastructural studies showed perineurial cell characteristics, including slender cytoplasmic processes, numerous pinocytotic vesicles, occasional tight junctions, and discontinuous surface basement membrane. Perineuriomas featuring marked stromal sclerosis often occur in the hands of young adults, as has recently been reported; the authors considered it a clinicopathologic variant. Herein we report two sclerotic perineuriomas, one a small tumor of the thumb and the other a centrally situated, massive pelvic tumor with sclerosis and degenerative cytologic atypia. We believe that exaggerated collagen deposition in perineuriomas, occasionally associated with degenerative nuclear changes, is not a manifestation of a unique, clinically relevant tumor variant but rather a manifestation of chronicity akin to that seen in "ancient schwannoma" and "atypical neurofibroma."