Background: Carcinosarcomas, a malignancy consisting of squamous cell carcinoma with sarcomatous features, are extremely rare and aggressive tumor of the vulva. Including this case, there are 17 cases reported in the literature. Risk factors for this entity are poorly understood.
Case report: We describe the case of a rapidly growing primary vulvar carcinosarcoma developing in an 84-year-old woman. The patient had previously received pelvic radiation for a squamous carcinoma of the anal canal. The excised vulvar tumor showed a superficial squamous carcinomatous element, associated vulvar intraepithelial neoplasia, and a transition into deeper sarcomatous component. By immunohistochemistry, the carcinomatous component was positive for keratins and negative for vimentin and smooth muscle actin, whereas the sarcomatous component was negative for keratins and positive for vimentin and smooth muscle actin. The patient was treated with hemivulvectomy with sentinel lymph node dissection followed by limited postoperative chemotherapy. The FIGO stage of the vulvar cancer was stage IB (T1 N0 M0), but even with this low stage, the patient had recurrence 17 months after treatment and died of her disease 8 months later. We compared age and stage at presentation, treatment, disease-free survival, and overall survival of our case to other reported vulvar carcinosarcomas.
Conclusions: Vulvar carcinosarcomas are poorly characterized aggressive tumors with poor outcome. This is the first case reported that points to previous radiation exposure as a possible etiologic agent for this lesion.