Cutaneous Sclerosing Perineurioma

Sclerosing perineurioma: case report and literature review

“…Consequently, this marker is useful for the differential diagnosis with dermatofibrosarcoma protuberans, fibromyxoid sarcoma, desmoplastic fibroblastoma, or fibromatosis [14]. Perineurioma is also characteristically positive for vimentin and there have been cases reported with positivity for cytokeratins, CAM 5.2, muscle-specific actin, collagen IV, laminin, GLUT-1, CD99, and CD34 [3,8,9,11,15]. In our patient, sclerosing perineurioma was positive for EMA and claudin-1, and negative for S100 protein.…”

“…Perineuriomas are uncommon tumors derived from perineural cells. They are usually located on the extremities of young adults [1][2][3], although there have also been reports in children [4]. Other body locations occasionally reported include the oral cavity [5,6] and scrotal region [7].…”

“…Two main variants have been identified: an extremely rare intraneural form and a more common extraneural or soft tissue variant [2,8]. A sclerosing extraneural variant was first described in 1997 [9]; since then less than 50 cases of this presentation have been documented [3,10]. Sclerosing perineurioma characteristically occurs on the hands as a single, asymptomatic papule or nodule of fibrous consistency, which is often confused with dermatofibroma [1][2][3]9].…”

“…A sclerosing extraneural variant was first described in 1997 [9]; since then less than 50 cases of this presentation have been documented [3,10]. Sclerosing perineurioma characteristically occurs on the hands as a single, asymptomatic papule or nodule of fibrous consistency, which is often confused with dermatofibroma [1][2][3]9]. Non-acral [5,6,11], multiple, and bilateral presentations have also been described [12,13].…”

“…Histologically, sclerosing perineurioma presents as a well-circumscribed, scantly cellular nodule located in the dermis or hypodermis. The lesion exhibits a variable number of small epithelioid and fusiform cells, which are arranged in an onion bulb-like and trabecular growth pattern, within a dense collagen stroma [2,3,9]. Histologically, the lesion must be distinguished from other sclerosing and/or fusocellular tumors.…”