Cutaneous lipomatous sclerosing perineurioma

Sclerosing perineurioma is a variant of extraneural soft tissue perineurioma that occurs in the distal extremities of young adults. They are composed of small, plump, spindle-shaped, or epithelioid perineurial cells that coexpress epithelial membrane antigen (EMA), glut-1, claudin 1, collagen type IV, laminin, and are negative for S-100 protein. Once locally excised, sclerosing perineurioma does not recur or metastasize. Adipocytic component has been observed occasionally in peripheral nerve sheath tumors. In particular, only one report has previously detailed histopathological features of a superficial soft tissue perineurioma harboring lipomatous areas. Herein, we report a unique case of sclerosing perineurioma showing adipocytic component. Knowledge of this phenomenon is important to reach a correct diagnosis and to avoid unnecessary aggressive local excision.