Primary adnexal adenocarcinoma of the vulva: A diagnosis of exclusion based on location, immunohistochemistry, and pattern of spread.

► Vulvar adenocarcinomas are rare comprising less than 0.1% of primary malignancies of the vulva. ► The diagnosis of primary vulvar adenocarcinomas remains a challenge due to its rarity, variation in histological appearance, and limited literature. ► The basis of the diagnosis includes morphology, immunohistochemistry, clinical history and pattern of spread.