Metastatic adenoid cystic carcinoma of the Bartholin Gland: Institutional cases and literature review of a rare gynecologic malignancy

• Metastatic spread of Bartholin gland adenoid cystic carcinoma synthesized. • Two institutional cases with integrated molecular findings. • Pulmonary metastasis identified as the most common distant site. • Delayed recurrence supports need for prolonged surveillance. To contextualize two institutional cases of Bartholin gland adenoid cystic carcinoma with biopsy-confirmed distant metastases within the broader published literature and to delineate clinical, pathologic, and molecular patterns associated with disease recurrence and dissemination. A retrospective review was performed of all Bartholin gland adenoid cystic carcinoma cases treated at our institution, supplemented by a comprehensive literature search of PubMed and Embase through 2025. Articles were included if they reported histologically confirmed Bartholin gland adenoid cystic carcinoma with documented distant metastasis. Extracted variables included patient demographics, pathology, treatment, molecular findings, metastatic sites, and outcomes. Fifteen cases of metastatic Bartholin gland adenoid cystic carcinoma were identified across eleven publications, in addition to two institutional cases. Most patients initially presented with localized disease managed surgically, often with adjuvant radiotherapy for positive margins or perineural invasion. The lung was the most common site of metastasis, followed by bone, liver, and brain. Time to distant recurrence ranged from 6 months to over 15 years. MYB or MYBL1 rearrangements were identified in most tumors, aligning Bartholin gland adenoid cystic carcinoma molecularly with salivary adenoid cystic carcinoma. Chemotherapy regimens were heterogeneous and primarily platinum-based, resulting in transient disease stability. Although Bartholin gland adenoid cystic carcinoma typically follows a locoregional course with favorable short-term outcomes, distant metastasis can occur years after initial treatment. Pulmonary involvement is most common, and prolonged surveillance is warranted. Molecular profiling may provide insight into disease behavior and inform future therapeutic strategies.