Primary adenoid cystic carcinoma of sweat glands in vulva: report of an unusual case and review of the literature.

Adenoid cystic carcinoma arising from the vulvar sweat glands is a rare malignancy of the female genital tract. We report a case of adenoid cystic carcinoma of sweat glands occurring in the left labia majora of a 52-year-old female patient. The patient underwent radical hemivulvectomy and left inguinal lymph node dissection with negative surgical margins and negative inguinal lymph node metastasis. Then, four episodes of combined chemotherapy without further radiotherapy were given. However, the tumor recurred after 3 months. Currently, the patient has been followed up for 2 years with no distant metastasis. According to our experience, although the tumor has a high tendency of local recurrence after resection, an acceptable survival time of the patient can be achieved with primary surgery.