Syringomatous eccrine carcinoma of the vulva

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 lesions of the vulva should be biopsied.