Primary malignant glandular tumor of the vulva: A three-case series and review of literature.

Vulvar cancer has an incidence of 2-3 per 100,000 women and accounts for 5% of all female genital tract (FGT) malignancies. About 90% of them are squamous cell carcinoma (SCC). Primary malignant glandular tumors of the vulva (PMGTV) are rare, diverse tumors with different prognoses and differentials. We present three cases of PMGTV diagnosed at our institution over 11 years (2013-2023) in the present study. Hospital intranet and medical records provided required clinical, histopathology, and immunohistochemistry (IHC) data. There were only three reported cases of PMGTV. The most common presentation was a painful mass growing rapidly. Most of the tumors were high-grade, and IHC was required for a definite diagnosis. A spectrum of variable entities was diagnosed, namely, adenoid cystic carcinoma (ADC), adenocarcinoma intestinal type, and adenocarcinoma of mammary gland type (AMGT). PMGTVs are rare entities and resemble morphologically and immunophenotypically tumors of other systemic sites, making exclusion of any primary malignancy mandatory. A large panel of immunohistochemistry is needed to typify them. Due to the rarity of the disease, no definite treatment guidelines are present.