Adenoid cystic carcinoma (ACC) of the Bartholin gland is a rare gynaecological entity. Despite its slow growth and inconspicuous presentation, vulvar ACC has a propensity for perineural invasion and is therefore associated with high local recurrence rates. We report a case of vulvar ACC in a 61-year-old woman with a prolonged swelling of the Bartholin gland. This patient presented with pulmonary metastases at the moment of histological diagnosis. The vulvar and the pulmonary lesions showed identical histology. Despite a history of human papilloma virus (HPV)-related usual type vulvar intra-epithelial neoplasia and cervical squamous cell carcinoma, the vulvar ACC was negative for both p16 immunohistochemistry and HPV-DNA. We conclude that HPV is not involved in the pathogenesis of pure ACC of the Bartholin gland. Additionally, we advocate a low threshold for performing biopsies of vulvar swellings in women aged >40 years, to rule out malignancy and to prevent diagnostic delays.

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Keywords Adenoid cystic carcinoma, Bartholin gland, Human papilloma virus, Lung metastasis, p16, Vulva
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Journal Pathology Research and Practice
Nieuwenhuyzen-de Boer, G.M, Dasgupta, S. (Shatavisha), Ewing, P.C, & van Bockstal, M. (2020). Adenoid cystic carcinoma of the Bartholin gland is not HPV-related: A case report and review of literature. Pathology Research and Practice. doi:10.1016/j.prp.2020.152968