Glandular and Other Malignancies of the Vulva

Glandular neoplasms of the vulvar are uncommon, and this chapter focuses on those entities that are particularly relevant with emphasis on confusing areas and potential pitfalls. Critical issues when managing Paget disease include ensuring the lesion arose in the vulva (versus anal region) and a clear understanding of the significance of invasion. Other adenocarcinomas, although rare, must be scrutinized to address potential origins, whether in the adnexal structures (Bartholin gland), mammary type tissue, cloacal remnants, or urethral mucosa. Adenoid basal cell carcinomas can be confused with adenoid cystic carcinomas, a potentially significant misdiagnosis. The specter of an exceeding rare monophasic (or nearly so) synovial sarcoma masquerading as a hidradenoma, primary adnexal or metastatic carcinoma carries with it treatment implications. Finally, neuroendocrine carcinomas (such as, Merkel cell carcinoma) round out this array of uncommon neoplasms, presenting its own challenges in diagnosis and management.