Adenoid cystic carcinoma of the Bartholin's gland: case report and systematic review of the literature.

Adenoid cystic carcinoma (ACC) of the Bartholin's gland is a rare malignancy of the female genital tract. Seventy-nine cases have been reported in the literature. A 40-year-old women presented to our clinic with twice locally recurrent ACC of Bartholin's gland of the left vulva despite hemivulvectomy. Adjuvant radiotherapy was delivered after the third resection. There was no local recurrence after three years follow-up, although she developed lung metastases that remain under control with oral cyclophosphamide. Our case confirms the literature review, which indicates a slow growing tumor with frequent local recurrences (30%) and distant metastases (31%). Adjuvant radiotherapy and/or chemotherapy should be considered in the management of ACC of Bartholin's gland.