Syringomatous Eccrine Carcinoma of the Vulva

Abstract

Abstract Background: Skin adnexal neoplasms of the vulva are uncommon, and malignant adnexal neoplasms of the vulva are rare. A case of syringomatous eccrine carcinoma (SEC) arising in the perineum is presented, and the literature reviewed. Case: A woman in her mid-70s presented with a perineal mass. After biopsy confirmation of the diagnosis, she underwent wide local excision of a 6.0 × 5.0 × 3.0 cm solid mass. The lesion came within 2 mm of the resection margin, and had lymph vascular space involvement. She was subsequently referred for radiation therapy. Results: Pathology testing showed that the multinodular yellow–tan lesion had eroded through the surface epithelium. Histologically, the tumor was composed of nests and sheets of poorly differentiated epithelial cells. At the periphery of the tumor, a spray pattern of keratinizing infiltrating eccrine ducts confirmed the diagnosis of SEC. Conclusions: Nonsquamous malignancy of the vulva should be considered when there are vulval lesions. All suspicious...