17. Benign and Malignant Conditions of the Vulva and Vagina Flashcards
female pseudohermaphroditism is caused by masculinization in utero of the female fetus, due to endogenous hormones from congenital adrenal hyperplasia, or androgen secreting tumors, obvious abnormalities include clitoromegaly, hypospadiac urethra meatus and malpositioned vaginal orifice. What results from mosaicism and can occur with varying degrees of virulization and mullerian development (androgen insensitivity syndrome)?
Male pseudohermaphrodistism
Androgen insensitivity is caused by genetic deficiency in androgen receptors 46XY, results in external female phenotypic development, most commonly xlinked recessive, testes are undescented, mullerian inhibiting sequence is produced by the 46xy which results in lack of mullerian duct development, ambiguous genitalial can be seen in partial androgen insensitivity. What occurs when child has both male and female development externally and internally?
TRUE hermaphroditism
Labial agglutination is treated w estrogen cream and massage to separate the labia majora, fox fordyce disease is severe pruritic raised yellow retention cyst in axilla and labia majora and minora resulting from keratin plugged inflammation of apocrine glands, what are located beneath the epidermis and are mobile, nontender, slow growing, most requiring no treatment?
Inclusion cyst- MC type of genital cysts- develop when hair follicles become obstructed
What is a rare condition in which one or more of the minor vestibular glands becomes infected, lesions are 1-4mm erythematous dots that are EXTREMELY tender, characterized by severe introtial dyspareunia and occasionally vulvar pain, can try topical estrogens/hydrocorstisone or surgical therapy may be required?
Vulvar Vestibulitis (vestibular adenitis)* KNOW
Fibromas are the MC benign solid tumors of the vulva, are slow growing ranging from 1-10 cm, can be gigantic. What cyst is caused by inflam blockage of sebaceous gland ducts, small smooth nodular masses usually on inner surface of labia minora/majora- contain cheesy secaceous material?
Sebaceous Cyst
Atrophic vaginitis is due to loss of estrogen (menopause/surgical) exam reveals atrophic external genetalia, minor regresses and majora shrinks, loss of vaginal rugae, vagina introitus constrictions, treatment via topical estrogen. What is local thickening of epithelium that results in prolonged itch-scratch cycle, exam reveals white red thickened leathery raised skin, bx reveals elongated rete ridges, hyperkeratosis of keratin layer, tx w steroids?
Lichen Simplex Chornicus (Squamous cell hyperplasia)
Lichen sclerosis is found on vulva of menopausal women, cause gential structural abnormalities, some progress to SCC, sx include pruritus, dyspareunia, and burning pain, examination reveals THIN white inelastic skin w crinkled tissue paper appearance- ONION skin, cigarette paper, parchement like… bx reveals thin epithelium and loss of rete ridges and inflammatory cells lining the?
basement membranes
tx w clobetasol
Lichen sclerosis is associated with loss of labia minora, shrinking or loss of labia majora, severe constriction of the introitus, and clitoriis can become inverted/trapped- hyperkeratosis is seen but epidermis is thinner than normal, striking feature of lichen sclerosis is presence of hyaline zone in the superficial dermis, due to edema and degeneration of collagen and elastic fibers of the?
dermis
Lichen planus are purplish polygonal papules that appear in erosive form, may involve vulva, vagina and mouth, sx include vulvar burning, severe insertional dyspareunia, tx topical roids. what occurs after birth (vagina) a bluging membrane like structure may be noticed in vaginal opening and can block egress of mucus, if not detected till after menarche, it can appear as thin dark bluish structure?
Imperforate Hymen (no opening)
Transverse vaginal septum is MC found in upper and middle thirds of vagina , may become apparent when intercourse is impeded, midline longitudinal vaginal septum creates a double vagina- associated with duplication anomalies of uterine fundus. What is when vag wall consists of islands of columnar cells in normal squamous epithelium due to exposure to DES?
Adenosis
Vaginal agenesis is when there is an abscence except for most distal protion, rokintansky-kuster-kauser syndrome= mullerian agenesis, if utereus is absent but fallopian tubes are spared. What is the MC vulvovaginal tumor less than 3cm, asymptomatic, usually unilateral swelling, need to bx in women 40+ to rule out bartholins carcinoma?
Bartholin’s CYst
What is the MC neoplasm of the vulva?
Squamous cell Carcinoma
Vulvar intrapeithelial neoplasia is used to denote high grade squamous lesions: 1) usual VIN- associated with HPV 16/smoking/IC status 2) differentiated VIN is NOT associated with HPV and is more commonly associated with vulvar derm conditions such as lichen sclerosis (older people)-pruritis is MC symptom, tx with?
surgical excision
Second MCC of vuvlar cancer is melanoma, mc in postmenopausal white women with lesions noted on the labia minora and clitoris, what carcinoma is a variant of SCC and metastasis is rare, lesions are cauliflower like in nature and can be confused with condyloma, ***RADIATION IS CONTRAINDICATED because may induce anaplastic transformation?
Verrucous Carcinoma
what is a rare vaginal cancer that presents as a mass of grape like polyps protruding from the vagina, histologically the tumor is embryonal cells, mean age is 2-3 years, treatments consist of surgical resection, chemo and radiation?
Sarcoma Botryoides (Embyonal Rhabdomyosarcoma)
