Adenoid Cystic Carcinoma of Bartholin's Gland: A Case Report with Emphasis on Surgical Management

Objective: Rare disease Background: Adenoid cystic carcinomas of Bartholin's gland are rare among gynecological malignancies, accounting for 0.1% to 7% of vulvar carcinomas and 0.001% of all female genital tract malignancies. There are no specific guide-lines regarding treatment recommendations; therefore, they are commonly treated like vulvar cancer. Case Report: We present the case of a 42-year-old premenopausal woman with an adenoid cystic carcinoma of Bartholin's gland diagnosed upon biopsy of a palpable, predominantly vaginally located mass causing foreign-body sen-sation, vaginal pain, and extreme dyspareunia. The adenoid cystic carcinoma of Bartholin's gland was treated by radical resection in an extensive interdisciplinary surgical approach including bilateral inguinal lymph node dissection, partial posterior colpectomy, amputation of the rectum, and creation of a descendostomy, as well as reconstruction of the vagina and defect coverage using flap plastic. Conclusions: With the presentation of this case, we propose a possible therapeutic approach to adenoid cystic carcinomas of Bartholin's gland with emphasis on surgical management. Especially in young patients, we recommend primary radical surgery with the objective to obtain negative resection margins. However, additional data on the adenoid cystic carcinoma of Bartholin's gland is needed to better understand its biological behavior and thus optimize and standardize treatment. The role of systematic inguinal-femoral lymphadenectomy and adjuvant and neoadjuvant treatment modalities need further evaluation.