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Objective: Adenoid cystic carcinoma (ACC) of Bartholin's gland s a distinctive entity of vulvar malignancy, accounting for 10-15% of Bartholin's gland carcinomas. The purpose of this paper is to report our diagnosis and surgical treatment of a case of primary Bartholin's gland carcinoma presenting as a Bartholin's cyst. Case Report: A 49-year-old woman was referred due to the increasing size of a posterior vulvar lump despite aspiration. Histology of the biopsy specimen confirmed ACC of Bartholin's gland. The patient was treated using radical vulvectomy with inguinal lymph node dissection. No evidence of local recurrence or distant metastasis was noted up to 18 months after surgery. Conclusion: Although uncommon, Bartholin's gland carcinoma should be suspected when excess induration or necrotic debris s present or when a Bartholin's cyst or abscess is unresponsive to conventional therapy, especially in perimenopausal and postmenopausal women.

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