Carcinosarcoma is a well-recognized tumor even if it is an uncommon entity. Neoplasms usually occur in the oral cavity, pharynx, esophagus, larynx and skin, and have been rarely documented in the female genital tract. This case reports a patient with a diagnosis of vulvar carcinosarcoma that has been treated with radical vulvectomy and a left inguinal lymphadenectomy but she died two months later of progressive disease. Because of the extreme rarity and severe prognosis of the tumor, we believe that this is a useful addition to the literature and might serves as a reminder to physicians that a multidisciplinary approach for management should be undertaken for treatment.
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