We report the case of a 51-year-old man with an asymptomatic indurated plaque on the chest wall that was surgically excised and submitted for histopathological examination. Microscopically, the dermis was occupied by a neoplastic process with spindle-shaped cells arranged in longitudinal fascicles with cytologic atypia; the abundant sclerotic stroma was composed of hyaline material. Tumoral cells showed immunohistochemical reactivity to smooth muscle markers. The diagnosis was desmoplastic leiomyosarcoma of the skin. This unusual entity is identified and discussed, and we review the literature.
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