A clinicopathologic and immunohistochemical study of 7 cases of sclerosing perineurioma.

Abstract

Sclerosing perineurioma is a relatively rare tumor that has remained widely unknown since first reported by Fetsch in 1997. To our knowledge, no large or small series of claudin-1 in sclerosing perineurioma has been confirmed to date. We collected 7 new cases of sclerosing perineurioma. Six patients were female, and 1 was male. The patients' age ranged from 15 to 58 years (mean, 36.6 years; median, 42.0 years). The primary reason for consultation at the outpatient clinics was a slowly enlarging mass. The preoperative durations were available for 4 of the 7 cases and ranged from 2 to 7 years. Six tumors were located in the fingers, and the other tumor was found in the palm. The sizes ranged from 4 to 12 mm in diameter (mean, 6.7 mm; median, 6.0 mm). Microscopically, all tumors were nodular lesions, with sclerotic stroma involving reticular dermis primarily consisting of small oval epithelioid cells and plump spindle cells, scattered and arranged in corded, trabecular, reticular, and/or whorled growth patterns. The neoplastic cells were immunoreactive for epithelial membrane antigen (7 of 7) and glucose transporter 1 (7 of 7). The periodic acid-Schiff reaction and positive immunostaining for type IV collagen were observed directly adjacent to the lesional cells in all cases (6 of 6). Claudin-1 immunostaining was positive in all 7 cases, suggesting that claudin-1 may serve as a helpful diagnostic marker for sclerosing perineurioma.