Vulva Pathology PDF

## VULVA ### I. BASIC PRINCIPLES * Anatomically includes the skin and mucosa of the female genitalia external to the hymen (labia majora, labia minora, mons pubis, and vestibule) * Lined by squamous epithelium ### II. BARTHOLIN CYST * Cystic dilation of the Bartholin gland * One Bartholin gl...

## VULVA ### I. BASIC PRINCIPLES * Anatomically includes the skin and mucosa of the female genitalia external to the hymen (labia majora, labia minora, mons pubis, and vestibule) * Lined by squamous epithelium ### II. BARTHOLIN CYST * Cystic dilation of the Bartholin gland * One Bartholin gland is present on each side of the vaginal canal and produces mucus-like fluid that drains via ducts into the lower vestibule. * Arises due to inflammation and obstruction of gland * Usually occurs in women of reproductive age * Presents as a unilateral, painful cystic lesion at the lower vestibule adjacent to the vaginal canal ### III. CONDYLOMA * Warty neoplasm of vulvar skin, often large * Most commonly due to HPV types 6 or 11 (condyloma acuminatum); secondary syphilis (condyloma latum) is a less common cause. Both are sexually transmitted. * Histologically, HPV-associated condylomas are characterized by koilocytes (hallmark of HPV-infected cells). * Condylomas rarely progress to carcinoma (6 and 11 are low-risk HPV types). ### IV. LICHEN SCLEROSIS * Characterized by thinning of the epidermis and fibrosis (sclerosis) of the dermis * Presents as a white patch (leukoplakia) with parchment-like vulvar skin * Most commonly seen in postmenopausal women; possible autoimmune etiology * Benign, but associated with a slightly increased risk for squamous cell carcinoma ### V. LICHEN SIMPLEX CHRONICUS * Characterized by hyperplasia of the vulvar squamous epithelium * Presents as leukoplakia with thick, leathery vulvar skin * Associated with chronic irritation and scratching * Benign; no increased risk of squamous cell carcinoma ### VI. VULVAR CARCINOMA * Carcinoma arising from squamous epithelium lining the vulva * Relatively rare, accounting for only a small percentage of female genital cancers * Presents as leukoplakia; biopsy may be required to distinguish carcinoma from other causes of leukoplakia. * Etiology may be HPV related or non-HPV related. * HPV-related vulvar carcinoma is due to high-risk HPV types 16 and 18. * Risk factors are related to HPV exposure and include multiple partners and early first age of intercourse; generally occurs in women of reproductive age * Arises from vulvar intraepithelial neoplasia (VIN), a dysplastic precursor lesion characterized by koilocytic change, disordered cellular maturation, nuclear atypia, and increased mitotic activity * Non-HPV related vulvar carcinoma arises, most often, from long-standing lichen sclerosis. * Chronic inflammation and irritation eventually lead to carcinoma. * Generally seen in elderly women (average age is > 70 years) ### VII. EXTRAMAMMARY PAGET DISEASE * Characterized by malignant epithelial cells in the epidermis of the vulva * Presents as erythematous, pruritic, ulcerated vulvar skin * Represents carcinoma in situ, usually with no underlying carcinoma * Paget disease of the nipple is also characterized by malignant epithelial cells in the epidermis of the nipple, but it is almost always associated with an underlying carcinoma. * Must be distinguished from melanoma, which rarely can occur on the vulva * Paget cells are PAS+, keratin+, and S100-. * Melanoma is PAS-, keratin-, and S100+.