Carcinosarcoma ex eccrine spiradenoma of the vulva: report of the first case

Carcinosarcoma is exceedingly rare in the vulva. We describe a case of carcinosarcoma in a 67-year-old female patient who presented with recent enlargement and pain of a vulval nodule noted for 15 years. The excised tumor showed intermixed carcinomatous (adenocarcinoma and anaplastic carcinoma) and sarcomatous elements (osteosarcoma, chondrosarcoma, and leiomyosarcoma), which focally merged with several lobules of typical eccrine spiradenoma. The inguinal lymph nodes showed metastasis of the carcinomatous component only. This case represents the first reported case of vulval carcinosarcoma of the skin adnexal origin, and has to be distinguished from sarcomatoid carcinoma of epidermal origin because of a probable more aggressive behavior.