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Bartholin gland carcinoma (BGC) is a rare malignancy, comprising 3–7% of vulvar cancers and <1% of gynecologic tumors. Due to its low incidence, high-level evidence is lacking, and management is largely extrapolated from vulvar cancer guidelines. This comprehensive narrative review synthesizes current evidence on BGC, emphasizing histotype-specific features, diagnostic criteria, molecular profiling, and treatment strategies. The three most common subtypes are squamous cell carcinoma, adenoid cystic carcinoma (AdCC), and adenocarcinoma. HPV-associated tumors tend to occur in younger women and carry favorable prognoses. Accurate diagnosis requires exclusion of metastases and integration of clinical, imaging, and immunohistochemical data, including p16/HPV for squamous tumors, MYB/MYBL1 fusions for AdCC, and CK20/CDX2/SATB2 for intestinal-type adenocarcinoma. Approximately 50% of cases are diagnosed at an advanced stage due to misclassification as benign cysts or abscesses. Nodal metastasis occurs in >40% of cases, with histotype influencing prognosis. Adenocarcinoma and node-positive disease independently predict worse survival. Treatment hinges on complete surgical excision with 2–3 mm margins, bilateral groin evaluation, and histology-tailored adjuvant therapy. Emerging data support the use of immune checkpoint inhibitors in squamous BGC and targeted agents (e.g., mTOR/CDK4/6 inhibitors) in adenocarcinoma. We propose a practical molecular testing algorithm and highlight the urgent need for prospective, multinational collaboration to establish BGC-specific guidelines.

Tumors affecting Bartholin’s gland are considered rare in human medicine; there are few reports in the veterinary literature, with descriptions occurring only in cows. This article described the clinical and pathological findings associated with Bartholin’s gland adenoma in a goat. Clinically, a 7-year-old pregnant Saanen goat presented bilateral enlargement of the vulva that did not regress spontaneously after parturition. Grossly, these vulvar masses were multilobulated, contained cystic areas from which oozed a whitish fluid. Histopathology revealed an adenoma characterized by the proliferation of irregularly shaped neoplastic epithelial cells that formed tubular to glandular-like structures. These neoplastic cells demonstrated moderate anisokaryosis and evident nucleoli. The intratumoral proliferation index (PI) was estimated by immunoreactivity with the protein ki-67. Further, the glandular-like structures produced a Periodic Acid-Schiff positive secretion. A diagnosis of Bartholin’s gland adenoma was established due to the anatomic location of the neoplastic growths, the histopathological features, and the PI of the tumor. RESUMO: Tumores que afetam a glândula de Bartholin são considerados raros em humanos e há poucos relatos na medicina veterinária, sendo descrito somente em vacas. Este artigo descreve os achados clínicos e patológicos associados ao adenoma na glândula de Bartholin em uma cabra. O animal da raça Saanen, com sete anos de idade e gestante apresentava aumento de volume bilateral na região da vulva, que não regrediu após o parto. Macroscopicamente, a massa era multilobulada, com presença de vários cistos preenchidos por uma secreção esbranquiçada. A histopatologia revelou um adenoma, caracterizado por proliferação de células epiteliais de formato irregular que formavam estruturas tubulares semelhantes à glândulas. Estas células neoplásicas apresentavam anisocariose moderada e nucléolos evidentes. O índice de proliferação celular intratumoral (PI) foi estimado por imunorreatividade à proteína ki-67. Além disso, a secreção produzida por essas estruturas do tipo glandular, foi positiva na coloração do Ácido Periódico-Schiff. O diagnóstico de adenoma da glândula de Bartholin foi estabelecido pela localização anatômica dos tumores neoplásicos, características histopatológicas e à PI do tumor.