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DISEASES OF THE VULVA AND VAGINA Vulvar disorders fall into the following main categories: Malformations Infectious inflammatory Immunologic Neoplastic etiologies Many inflammatory dermatologic diseases that affect skin elsewhere on the body may also occur on the vulva: Psoriasis Eczema Allergic dermatitis BARTHOLIN CYST AND ABSCESS Bartholin glands are located at the entrance to a woman's vagina, one on each side The duct is lined with transitional epithelium. The peripheral acini are single-layered columnar cells with clear cytoplasm. Bartholin cyst Bartholin cysts form when the ostium of the duct becomes obstructed, leading to distention of the gland or duct with fluid Usually secondary to nonspecific inflammation or trauma lined by the metaplastic squamous epithelium Bartholin abscess Result from a primary gland infection or infected cyst Patients = acute, rapidly progressive vulvar pain Abscesses are polymicrobial and rarely attributable to sexually transmitted pathogens most common = are opportunistic bacteria such as Staphylococcus species, Streptococcus species, and most commonly, Escherichia coli. LEUKOPLAKIA. Definition: lesions of the vulva presents as a whitish patch or plaque-like mucosal thickening that may produce itching (pruritus) and scaling Represent a variety of benign, premalignant, or malignant: 1. Lichen simplex chronicus (squamous cell hyperplasia): An idiopathic condition in which lichenification of the vulva is caused by persistent itching and scratching thickening of the skin, leathery or bark-like appearance of the skin Clinical presentation: erythematous, firm, rough plaques with exaggerated skin lines (lichenified plaques). Hyperpigmentation of lichenified plaques a person senses pruritus in a specific area of skin (with or without underlying pathology) and causes mechanical trauma to the point of lichenification Occurs in the genital and perianal regions, as well as the posterior neck, forearms and pretibial areas Etiology unknown but patients with underlying atopic dermatitis appear to be at increased risk A relationship is suspected between the central nervous system and inflammatory cell products in the perception of itch and ensuing skin changes in lichen simplex chronicus. Emotional tensions in predisposed subjects may play a key role in inducing a pruritic sensation, leading to scratching that can become self-perpetuating. Micro findings: Repetitive scratching or rubbing from irritants can result in squamous cell hyperplasia. Squamous cell hyperplasia is the hallmark of lichen simplex chronicum Epithelial thickening, expansion of the stratum granulosum, and significant surface hyperkeratosis Increased mitotic activity Leukocytic infiltration of the dermis is sometimes pronounced. The hyperplastic epithelial changes show no atypia Recent years: Some cases of lichen simplex chronicus in elderly women have been published associated with Human papillomavirus-negative invasive squamous carcinoma of the vulva in the adjacent skin 2. Lichen sclerosus: the skin = thin, whitened, wrinkled, and can cause itching and pain; most commonly occurs in the vulva, and in the anal region postmenopausal women Etiology: Genetic factors: families are genetically predisposed after experiencing trauma, injury, or sexual abuse. Autoimmune diseases: possibly extracellular matrix protein-1 (ECM-1) as antibodies to this protein; Other autoimmune conditions such as thyroid disease (about 20% of patients), pernicious anemia, vitiligo, and psoriasis are reported Clinical presentation: most common = vulvar itching; anal itching possible experience dyspareunia, dysuria, painful defecation and anal fissures Gross: the skin appears white, with no pigmentation, and very thin and wrinkled, classically referred to as a “cigarette paper” (or “parchment”) appearance It may progress and distort the appearance of the genital area as the labia minora become atrophic, fuse, disappear and bury the clitoris The vagina can become narrowed, and cracks, fissures, and thickened, scarred skin in the genital and anal area Morphology: thinning of the epidermis and disappearance of rete ridges, hydropic degeneration of the basal cells, superficial hyperkeratosis, and dermal fibrosis with a scant perivascular, mononuclear inflammatory cell infiltrate Lichen sclerosus and cancer increased risk for developing squamous cell carcinoma of the vulva 1. Vulvar intraepithelial neoplasia and vulvar carcinoma (VIN) precancerous lesions 5% of all female genital tract malignancies and occurs most frequently in women between the ages of 65 and 75; vast majority (approximately 90%) are squamous cell carcinomas other histologic lesions including melanomas, adenocarcinomas, basal cell carcinomas, and sarcomas Classification: two groups A. Warty carcinoma , Basaloid carcinoma high oncogenic risk HPVs (type 16) Basaloid carcinoma shows an infiltrating tumor characterized by nests and cords of small, tightly packed malignant squamous cells lacking maturation that resemble immature cells from the basal layer of the normal epithelium. The tumor may have foci of central necrosis Verrucous carcinoma is characterized by exophytic, papillary (warty) architecture and prominent koilocytic atypia (i.e. cellular features of HPV infection). Verrucous carcinoma is locally invasive but rarely metastasizes B. keratinizing squamous cell carcinoma not related to HPV infection. Vulvar carcinomas develop from a precancerous in situ lesion called vulvar intraepithelial neoplasia (VIN). 1. Classic VIN is associated with HPV infections, most often HPV type 16, and typically occurs in women between 30 and 40 with a history of multiple sexual partners. proliferation of small immature cells, nuclear atypia and increased mitoses, involving full thickness of the epidermis 2. simplex VIN postmenopausal age range not associated with HPV Simplex VIN has a stronger association with lichen sclerosus and lichen simplex chronicus Micro: marked atypia of the basal layer of the squamous epithelium with apparently normal epithelial maturation and differentiation in the superfical layers, thus the designation “differentiated VIN.” Presents as red and velvety, or white and elevated plaque-like lesion that usually involves labia majora. A. HPV-associated vulvar squamous cell carcinomas (classic) long-standing lichen sclerosus or lichen simplex chronicus. The mean age of the patients is 76 years simplex VIN initial lesion characterized this way Histologic examination reveals infiltrating nests and tongues of malignant squamous epithelium with prominent central keratin pearls 1. Verrucous carcinoma (histo) well-differentiated, low grade tumor exophytic mass resembling condyloma acuminatum. Its prognosis is excellent; slow regrowth may be a problem tumor has pushing borders into the underlying dermis, but does not show frank destructive invasion Shows verrucous architecture Papillary hidradenoma Vulva may contain tissue closely resembling breast (“ectopic breast”) and develop two tumors with counterparts in the breast: Papillary hidradenoma Extramammary Paget disease: characterized by infiltration of the squamous mucosa or adnexa by mucin-producing neoplastic cells; similar in its manifestations to Paget disease of the breast. Multipotential cells of epidermal basal layer that differentiate along glandular (sweat gland) lines Gross: Papillary hidradenoma in general is a sharply circumscribed nodule, most commonly on the labia majora or interlabial folds, and may be confused clinically with carcinoma because of its tendency to ulcerate. Histo: Identical in appearance to intraductal papillomas of the breast and consists of papillary projections covered with two layers of cells: the top columnar, secretory cells and an underlying layer of flattened myoepithelial cells; myoepi = characteristic of sweat glands and sweat gland tumors Extrammamary Pagent disease of the Vulva Elevated, scaling, pruritic, and red eczematous-appearing area, occurring usually on the labia majora. Micro: Epidermis contains large pale tumor cells (Paget cells) that form small solid nests or a continuous layer along the epidermal basement membrane Stains with periodic acid–Schiff (PAS), Alcian blue, or mucicarmine stains. Vulvar lesions are most frequently confined to the epidermis of the skin and adjacent hair follicles and sweat glands Prog: Paget disease is treated with wide local excision and shows a high recurrence rate. Frequently present beyond the margins of surgical excision Intraepidermal Paget disease may persist for many years, even decades, without invasion or metastases Malignant melanoma: Tend to have the same biologic and histologic characteristics as melanomas occurring elsewhere in the skin and are capable of widespread metastatic dissemination. Prognosis is linked principally to depth of invasion, with greater than 60% mortality for lesions invading deeper than 1 mm Diseases of the Vagina A. DEVELOPMENTAL ANOMALIES 1. Imperforate hymen the most frequent obstructive anomaly of the female genital tract; usually asymptomatic until a child reaches menarche; repaired with a simple incision 2. Vaginal agenesis absence of the uterus, proximal vagina, and, in some cases, the fallopian tubes. This anomaly has been recently termed Müllerian aplasia 3. Vaginal atresia the urogenital sinus fails to contribute to the inferior portion of the vagina; müllerian structures are usually normal but fibrous tissue completely replaces the inferior segment of the vagina 4. Double vagina failure of total fusion of the müllerian ducts and accompanies a double uterus (uterus didelphys); vaginal duplication and lack of absorption of the wall between the two ducts will leave a residual vaginal septum 5. Vaginal adenosis remnant of columnar, endocervical-type epithelium that during embryonal development extends from the endocervix and covers the ectocervix as well as the upper vagina and is subsequently replaced by the squamous epithelium advancing upwardly from the urogenital sinus. Small patches of unreplaced glandular epithelium may persist focally into adult life. It has been reported in 35% to 90% of women exposed to diethylstilbestrol (DES) in utero DES was shown to cause a rare vaginal tumor (clear cell adenocarcinoma of the vagina) arising in DES-related adenosis in girls and young women who had been exposed to this drug in utero red, granular areas contrasting with the normal pale-pink vaginal mucosa Micro: columnar mucinous epithelium indistinguishable from endocervical epithelium 6. Gartner duct cysts lesions found along the lateral walls of the vagina and derived from wolffian (mesonephric) duct rests VAGINAL INTRAEPITHELIAL NEOPLASIA AND SQUAMOUS CELL CARCINOMA Primary carcinoma an extremely uncommon cancer Almost all of these tumors are squamous cell carcinomas associated with high risk HPVs. The greatest risk factor is a previous carcinoma of the cervix or vulva Squamous cell carcinoma of the vagina arises from a premalignant lesion, vaginal intraepithelial neoplasia, analogous to cervical squamous intraepithelial lesions affects the upper posterior vagina, particularly along the posterior wall at the junction with the ectocervix. lesions in the lower two thirds of the vagina metastasize to the inguinal nodes, whereas upper lesions tend to involve the regional iliac nodes A. CLEAR CELL ADENOCARCINOMA upper vagina of children and young adults. history of intrauterine exposure to diethylstilbestrol (DES) 2/3rd of patients B. EMBRYONAL RHABDOMYOSARCOMA (Also called sarcoma botryoides) Infants and in children younger than 5 years of age and consists predominantly of malignant embryonal rhabdomyoblasts Divided into several histological subsets Embryonal most frequently observed Embryonal botryoid arise under the mucosal surface of body cavities such as the urinary bladder or vagina. Vaginal bleeding and discharge are the most common presenting symptoms. Spindle cell subtypes Alveolar Anaplastic Undifferentiated On physical exam polypoid mass resembling a bunch of grapes fills the vagina The tumor cells are crowded beneath the vaginal epithelium, in a so-called cambium layer, but in the deep regions they lie within a loose fibromyxomatous stroma that is edematous and may contain many inflammatory cells Can be mistaken for benign inflammatory polyps These tumors tend to invade locally and cause death by penetration into the peritoneal cavity or by obstruction of the urinary tract. Conservative surgery, coupled with chemotherapy, seems to offer the best results in cases diagnosed sufficiently early.
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