Adenoid cystic carcinoma of Bartholin’s gland (BG-ACC) is a rare vulvar
carcinoma which has its own unique characteristics, such as growing
slow, local invasion, perineural infiltration and high local recurrence
rates. Signs and symptoms are nonspecific. Perineural invasion may be
the reason for causing pain and high recurrence rates. There is no
consensus on treatments. We collect all current literatures about BG-ACC
to summarize the clinical features, histological features and treatment
options. Surgery is the cornerstone of treatments. Adjuvant chemotherapy
and radiation therapy may be beneficial in case of distant metastasis.
We also need more research in molecular targeting therapy.