PURPOSE: This article reports on a sclerosing perineal-perianal rhabdomyoma in an adult woman. A review of the literature disclosed no reference to this histological form of rhabdomyoma in women. METHODS AND RESULTS: We describe the case of a 48-year-old woman. At physical examination, a firm nodular lesion was identified in the left lateral perianal-perineal region. Histologically, it comprised rounded and elongated muscle fibers displaying no evident atypia, within a stroma exhibiting marked sclerosis and mild lymphoid infiltrate. At immunohistochemical examination, tumor cells stained for desmin, muscle-specific actin, and myogenin. CONCLUSIONS: Skeletal muscle rhabdomyomas are rare benign tumors, generally divided into 3 major categories depending on their clinical and morphological characteristics: fetal, adult, and genital. An unusual histological variant of the genital form has recently been reported in men, characterized by the presence of predominantly mature muscle fibers and marked sclerosis. We document such a lesion in a female patient for the first time.
PURPOSE: This article reports on a sclerosing perineal-perianal rhabdomyoma in an adult woman. A review of the literature disclosed no reference to this histological form of rhabdomyoma in women. METHODS AND RESULTS: We describe the case of a 48-year-old woman. At physical examination, a firm nodular lesion was identified in the left lateral perianal-perineal region. Histologically, it comprised rounded and elongated muscle fibers displaying no evident atypia, within a stroma exhibiting marked sclerosis and mild lymphoid infiltrate. At immunohistochemical examination, tumor cells stained for desmin, muscle-specific actin, and myogenin. CONCLUSIONS: Skeletal muscle rhabdomyomas are rare benign tumors, generally divided into 3 major categories depending on their clinical and morphological characteristics: fetal, adult, and genital. An unusual histological variant of the genital form has recently been reported in men, characterized by the presence of predominantly mature muscle fibers and marked sclerosis. We document such a lesion in a female patient for the first time.