Adenoid cystic carcinoma of Bartholinʹs gland:what is the optimal approach?

Aims and methods:We report the case of a locally advanced adenoid cystic carcinoma of Bartholinʹs gland which required posterior pelvic exenteration, radical vulvectomy and ablation of the ischiorectal fossa in order to obtain tumour clearance with negative margins. Including this case, only three pelvectomies have been performed for this disease. This is the first reported case in which a controlateral unsuspected intravulvar metastasis was found on histology.Results and conclusions:No consensus exists on the adequate surgical management of this particular disease. Nevertheless, a review of the literature and this reported case suggest that radical vulvectomy with negative margins should be preferred to wide local excision as the primary surgical procedure. It also suggests that inguinofemoral lymph-node dissection should only be performed when suspect lymph nodes are found at clinical examination.