Vulvar Adnexal Lesions A 32-Year, Single-Institution Review From Massachusetts General Hospital

Context.-Because the skin and modified mucosal surfaces of the vulvar region contain dense apocrine glands and anogenital mammary-like glands, in addition to eccrine glands and folliculosebaceous units, benign as well as malignant lesions derived from these adnexal structures are, not surprisingly, found in the vulva. However, their incidence occurring in the vulva has not been reported, to our knowledge. Objective.-To determine the incidence of various vulvar adnexal lesions. Design.-We performed a retrospective review (19782010) of the cases at our institution. Results.-A total of 189 vulvar adnexal lesions were identified. Most of these lesions were benign (133 of 189; 70%), with hidradenoma papilliferum being the most common, followed by syringoma and various types of cysts. Rare cases of tubular adenoma, poroma, spiradenoma, hidradenoma, cylindroma, sebaceoma, and trichoepithelioma were identified. Malignant adnexal neoplasms comprised the remaining 30% (56 of 189) of the cases. Extramammary Paget disease was the most common (49 of 56), and 29% (14 of 49) demonstrated an invasive component. Rare cases of basal cell carcinoma, sebaceous carcinoma, apocrine carcinoma, adenoid cystic carcinoma, and spiradenocarcinoma were identified. Conclusions.-In this retrospective review, we identified several benign entities that have not been previously reported on the vulva, namely pilomatricoma, poroma, spiradenoma, and sebaceoma. Hidradenoma papilliferum and extramammary Paget disease were the most common benign and malignant adnexal neoplasms, respectively. The spectrum of various vulvar adnexal lesions appears to reflect the frequency of the underlying glandular elements.