Introduction/Background Adenoid Cystic Carcinomas of Bartholin’s gland (ACCBG) are a rare entity, as they only represent 0.1% to 7% of vulvar carcinomas and 0.001% of all female genital tract malignancies. Because of their low frequency, no guidelines can be found regarding their treatment and usually they are treated as any other vulvar cancer.

Results A 47-year-old woman is referred to our public health centre to evaluate a long-term and painful bilateral vulvar lump. A bilateral tumourectomy was performed and histological examination informed of a bilateral ACCBG, pT4a.

Immunohistochemistry showed positivity for p63 and S100 in myoepithelial cells and for CD117 (c-kit) and CD43 in the internal layer. Proliferation index Ki67 reached 30% in some tumoral nuclei. The case underwent the interdisciplinary tumour board and thoracic-abdominal CT scan and pelvic MRI were performed. There was no distant dissemination but the persistence of tumoral image of the surgical margins and bilateral inguinal adenopathy.

The patient had to go under rescission and inguinal lymphadenectomy due to affected margins. A new histological examination showed dermis and soft tissue infiltration with perineural infiltration. No lymph nodes were found affected. Thereupon, she undertook adjuvant radiotherapy. Four years later, lymphadenopathy images persist despite no other signs of remaining illness.

Conclusion Bilateral ACCBG wouldn’t be the first diagnostic to come to mind if suspicion of Bartholinitis but to do a biopsy of BG may be a good option in long-term cases of recurrence. If not, rescission may need to be performed.

Disclosures The authors declare that they have no conflict of interest with respect to the author or publication of this article.