Adenoid cystic carcinoma of the Bartholin gland (BG-ACC) is a rare form of vulvar cancer. Literature reported approximately 350 cases of BG-ACC since 1864. Literature data and case reports suggested an aggressive nature with protracted clinical symptoms and a tendency for local recurrence despite adequate surgical excision with or without adjuvant radiotherapy. Survival rates of 71% and 59% are reported, respectively, at five and ten years. A comparative analysis of the immunohistochemical profile was performed with the homologous tumor of the salivary glands, and it was observed that the expression of various antigen in different morphological patterns of this neoplasia allowed some considerations about on its histogenesis that was hitherto never proposed.
