Adenoid cystic carcinoma of Bartholin’s gland is characterized by slow growth, local invasion, and sometimes distant metastasis. There is no consensus regarding the optimal treatment. We report herein a rare case of stage II adenoid cystic carcinoma of Bartholin’s gland that was successfully treated by wide local excision of the tumor and ipsilateral inguinal and pelvic lymphadenectomy. A 54-year-old Japanese woman was admitted complaining of painless, gradually increasing vulvar mass existing from ten years before. Local examination revealed a 3 × 2-cm hard mass in the left labium major. Pathological examination of subsequent excisional biopsy revealed adenoid cystic carcinoma originating in the Bartholin’s gland. A wide local resection of the tumor with inguinal and pelvic lymphadenectomy was performed. The tumor was completely excised with free margins. There was no metastasis in the resected lymph nodes. The patient was diagnosed as having stage II vulvar cancer according to the International Federation of Gynecologists and Obstetricians classification (1988). The patient is now healthy without evidence of recurrence at 5 years after surgery. Adenoid cystic carcinoma of Bartholin’s cyst at early stage can be treated by wide local excision as a primary surgery.

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