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

Abstract

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 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.

Invasive Paget Disease of the Vulva

“…The secondary noncutaneous forms are considered as epidermotropic metastasis originating from an underlying malignancy of the gastrointestinal tract (type 2), the urogenital tract (type 3), or an adenocarcinoma originating elsewhere. 4,5 Because of its rarity and unclear presentation features, EPDV is often diagnosed with delay, leading to the development of extensive lesions. Primary lesions follow an unpredictable course and, even though they are mostly slow-growing diseases with an indolent course, invasion can occur, and potential lymph node metastases or, rarely, hematogenous spread take place.…”

Tubular Apocrine Adenoma – A Case Report of Rare Sweat Gland Tumor

“…[6] The finding of a tubular apocrine adenoma in a female of middle age in our case is in concordance with what others have reported. [1][2][3][4][5][6][7]10,12,[14][15][16][17][18] Tubular apocrine adenoma is found most commonly on the scalp but lesions have also been described at a variety of other sites including the face, [6] eyelid, [18] axilla, [3] leg and genitalia. [4,11,17] However, Lee et al reported a case in the external auditory canal.…”

Vulvar Fibroadenoma: Case report and review of literature

“…Several forms of accessory breast has been classified by Kajava which depends on the presence of breast structures (nipple, areola, glandular tissue) and ranges from complete breast with nipple, areola and glandular tissue to polytheliapilosa (presence of only patch of hair). Japanese women have the highest incidence of accessory mammary tissue [3].…”