Extra-Acral Cutaneous/Soft Tissue Sclerosing Perineuriomas: An Underrecognized Entity in the Differential of CD34 Positive Cutaneous Neoplasms
MD Fox, BC Gleason, AB Thomas, TL Cibull. NorthShore University HealthSystem, Evanston, IL; Diagnostic Pathology Group, Sacramento, CA
Background: Perineuriomas are an uncommon group of tumors composed of perineurial cells of peripheral nerve sheath derivation. They have been further classified as soft tissue, intraneural, sclerosing, and reticular variants; each having distinct immunohistochemical and ultrastructural properties. Sclerosing perineuriomas have been reported to occur almost exclusively on the hands of young men. Histologically the cells are arranged in cords, trabeculae, and chains within a densely sclerotic stroma and the nuclei vary from spindled to round. We describe three examples of cutaneous/soft tissue sclerosing perineuriomas with extra-acral involvement.
Design: We recently encountered three extra-acral cutaneous/soft tissue perineuromas which demonstrated prominent sclerotic change. Each case was evaluated for the amount of sclerotic change, which was further classified as focal or diffuse. Additionally, all three cases were characterized immunohistochemically for expression of EMA, CD34, and S-100.
|Forearm||46||+ (focal)||+ (diffuse)||-||+ (focal)||Epithelioid/Spindle|
|Lower lateral leg||60||+ (diffuse)||+ (diffuse)||-||+ (diffuse)||Spindle|
|Upper lip||84||+ (diffuse)||+ (diffuse)||-||+ (diffuse)||Spindle|