Abstract
Background. A sarcomatoid squamous cell carcinoma of the vulva is a very rare malignancy of the female genital tract. It has been characterized as an aggressive disease typically associated with early development of local recurrences as well as distant metastases. Cases. A 77-year old woman presented to our hospital with an aggravated vulvar mass, complaining about pruritus and pain. The patient underwent a radical vulvectomy with bilateral inguinal lymph node dissection and bilateral pelvic lymph node sampling. The histopathologic examination was consistent with a sarcomatoid squamous cell carcinoma of the vulva with an identical microscopic and immunohistochemical appearance. The FIGO stage was IB and further management was decided to be radiotherapy. Conclusion. A sarcomatoid squamous cell carcinoma is a rare entity with an aggressive biological behavior. To date, there have been only a few cases of the disease reported in the literature. Therefore a more systematic collection and closer study of such cases would be helpful for achieving an early diagnosis and more effective therapeutic strategies in the future.