Benign and Malignant Conditions of the Vulva and Vagina Flashcards
How can ambiguous genitalia present?
- Clitormegly
- Clitoral agenesis
- Bifid clitoris
- Midline fusion of the labioscrotal folds
- Cloaca (no definite separation between the vagina and bladder
What is required for ambiguous genitalia?
- Careful examination
- PE, US, hormonal studies, karyotyping
What is the result of an infant with suboptimal development of penile or scrotal structures?
- Infant is assigned female
What is female pseudohermaphroditism?
- Masculinization in utero of the female fetus
What causes female pseudohermaphroditism?
- Endogenous hormonal milieu (congenital adrenal hyperplasia, ingestion of exogenous hormones, androgen secreting tumors of the mothers adrenals or ovaries)
What is seen in female pseudohermaphroditism?
- Clitoromegaly
- Hypospadiac urethral meatus
- Malpositioned vaginal orifice
- Internal genital organ development is normal
What is male pseudohermaphorditism?
- Results from mosaicism and can occur with varying degrees of virtualization and mullerian development
What causes androgen insensitivity?
- Genetic deficiency in androgen receptors
What is the karyotype of androgen insensitivity?
- 46 XY
- Most commonly an x-linked recessive disorder
What is the clinical presentation of an infant with androgen insensitivity?
- External female phenotypic development
- Testes are undescended
- Müllerian inhibiting substance is produced by the 46xy resulting in a lack of müllerian duct development (no uterus or fallopian tubes)
What is true hermaphroditisim?
- Rare
- Affected child has both male and female development externally and internally (combined ovotestes or separate gonads)
- Extent of masculinization depends on amount of functioning testicular tissue
How is labial agglutination treated?
- Estrogen cream is massaged on to separate the labia majora
What is Fox-Fordyce disease?
- Severe pruritic raised yellow retention cysts in the axilla, labia majora, and minor resulting from keratin plugged inflammation of apocrine glands
What are epidermal inclusion cysts?
- Located beneath the epidermis and are mobile, nontender, spherical, and slow growing
- Most common of genital cysts
- Develop when the hair follicle becomes obstructed; deeper portion swells to accommodate the desquamated cells
What are vulvar varicosities?
- Can enlarge and become painful in pregnancy
- Have characteristic blue color
What are urethral caruncles?
- Appear as a small fleshy red outgrowth at the distal edge of the urethra
What causes urethral caruncles in children?
- Spontaneous prolapse of the urethral epithelium
What causes urethral caruncles in postmenopausal women?
- Secondary to contraction of the hypoestrogenic vaginal epithelium resulting in everting of the urethral epithelium
What is vulvar vestibulitis?
- Rare condition in which one or more of the minor vestibular glands becomes infected
What is seen with vulvar vestibulitis?
- Lesions are 1-4 mm erythematous dots that are extremely tender
- Characterized by severe introital dyspareunia and occasionally vulvar pain
How is vulvar vestibulitis treates?
- Can try topical estrogens/hydrocortisone
- Surgical therapy may be required
What is a sebaceous cyst?
- Caused by inflammatory blockage of the sebaceous gland duct
- Small, smooth, nodular masses usually on inner surface of labia minora and majora
- Contain a cheesy sebaceous material
What are fibromas?
- Most common benign solid tumor of the vulva
- Slow growing, most range from 1-10 cm
What is a lipoma?
- Slow growing tumors composed of adipose cells
What is a hidradenoma?
- Rare lesion arising from sweat gland of the vulva
What is a syringoma?
- Eccrine gland tumor
What is a neurofibroma?
- From Von Recklinghausen’s disease
What is an angioma?
- Appear as multiple 2-3mm red lesions usually in fourth or fifth decade
What is a vulvar hematoma?
- Loculated collection of blood that collect following trauma (bike injury, birth trauma, or sexual assault)
What may be warranted with a vulvar hematoma?
- Close observation and occasional surgical exploration
What is female genital circumscision?
- More common in Africa and Eastern Asia countries
- Degree of anatomic change has an effect on infection risk, sexual function and vaginal delivery
What causes atrophic vaginitis?
- Menopause
- Surgical removal of uterus and ovaries
What will exam reveal for someone with atrophic vaginitis?
- Minora regresses and majora shrinks
- Loss of vaginal rugae
- Vaginal introitus constriction
What is the treatment for atrophic vaginitis?
- Topical estrogen
- May consider oral estrogen to prevent recurrence
What is lichen simplex chronicus?
- Local thickening of epithelium that results from a prolonged itch-scratch cycle
What is a symptoms of lichen simplex chronicus?
- Pruritus
What does exam reveal for lichen simplex chronicus?
- Reveals white or reddish, thickened, leathary, raised surface
- Looks similar to psoriasis
What does biopsy reveal for lichen simplex chronicus?
- Elongated rete ridges
- Hyperkeratosis of the keratin layer
What is the treatment for lichen simplex chronicus?
- Moderate strength steroid ointments with antipruritic agents
What is lichen sclerosis?
- Most frequently found on vulva of menopausal women
- Can cause genital structural abnormalities
What are some symptoms of lichen sclerosis?
- Intense pruritus
- Dyspareunia
- Burning pain
What does examination reveal for lichen sclerosis?
- Thin, white, inelastic skin with crinkled tissue paper appearance
- “Onion skin, cigarette paper, parchment like”
What does biopsy reveal for lichen sclerosis?
- Thin epithelium
- Loss of rete ridges and inflammatory cells lining the basement membrane
What is the treatment for lichen sclerosis?
- Clobetasol 0.05%
What could some women with lichen sclerosis develop?
- Squamous cell carcinoma of the vulva
What is seen on exam for lichen planus?
- Purplish, polygonal papules that may appear in an erosive form
What is vulvar-vaginal-gingival syndrome?
- When lichen planus involves the vagina, vulva, and mouth
What are the symptoms of lichen planus?
- Vulvar burning
- Severe insertional dyspareunia
What is the treatment for lichen planus?
- Topical and systemic steroids
What is psoriasis?
- Autosomal dominant inherited disorder
- On the vulva generally appears velvety but may lack the silver scaly patches found on flexor surfaces
What does eczema look like?
- More erythematous presentation
What is pemphigus?
- Autoimmune blistering disease involving the vulvovaginal and conjunctival areas
What is Behcet’s syndrome?
- Classically involves ulcerations in the genital, oral areas with uveitis
What is crohn disease?
- Primarily a GI disorder but vulvar ulcerations can occur due to fistulization
What are apthous ulcers?
- Superficial and painful
- More commonly found in mouth
What are decubitus ulcers?
- Can develop when chronic pressure is applied or secondary to tissue being moist secondary to urinary incontinence
What is acanthosis nigrcans?
- Most commonly found in the intertriginous area, vulva, axilla, or nape of neck
- Appears as a demarcated, brown pigmented thickened area in the superficial layers of the skin
What is acanthosis nigricans most commonly associated with?
- Insulin resistance and obesity but can be linked to other benign conditions and malignancy
What is contact dermatitis?
- Careful history may identify the specific irritant
- PE may reveal erythema, edema, excoriation, or ulceration
- May need biopsy
What is an imperforate hymen?
- After birth, a bulging, membrane-like structure may be noticed in the vaginal opening, can block the egress of mucus
- If not detected until after menarche, imperforate hymen can appear as a think dark bluish structure which entraps menstrual flow
What is a transverse vaginal septum?
- Most commonly found in the upper and middle thirds of the vagina often a small sinus tract or perforation will be present which allows the egress of menstrual flow
- May only become apparent when intercourse is impeded
What is a midline longitudinal vaginal septum?
- Creates a double vagina, a longitudinal septum can attach to the lateral wall thus creating a blind vaginal pouch
- These are usually associated with various duplication anomalies of the uterine fundus
What is vaginal agenesis?
- Most extreme vaginal anomaly with total absence of the vagina except for the most distal portion derived from the urogenital sinus
What is Rokitansky-Kuster-Hauser syndrome?
- Mullerian agenesis
- Uterus is absent but fallopian tubes are spared
What is adenosis?
- If the vaginal wall consists of islands of columnar cells in normal squamous epithelium
- Seen in women who have been exposed to DES in utero
What is gartner’s duct cyst?
- Arise from the remnant of the wolffian duct
- Vary in size from 1-5 cm and are found in the lateral walls of the vagina
- Most are asymptomatic and require no intervention
What is a urethral diverticula?
- Small 0.3 -3 cm sac like projections in the anterior vagina along the posterior urethra
- Can cause recurrent UTI, dysuria, and occasionally urinary leaking
- Urethral dilation or excision is treatment
What is an inclusion cyst?
- Result form infolding of the vaginal epithelium
- Located in the posterior or lateral wall in the lower third of the vagina
- Frequently associated with gynecologic surgery or lacerations from child birth
What is a bartholin’s cyst?
- Most common vulvovaginal tumor
- Less than 3 cm and is usually asymptomatic
- Usually unilateral swelling
- Need to biopsy in women 40+ to rule out Bartholin’s carcinoma
What is a bartholin’s gland abscess?
- Results from blockage and accumulation of purulent material
- Painful inflammatory mass arises
What are some treatment options for bartholin’s gland abscess?
- Word catheterization: Leave in 4-6 weeks which promotes an epithelialized tract for drainage of glandular secretions
- Marsupialization: creates a new ductal opening by everting the cyst wall onto the epithelial surface where it is sutured with interrupted absorbable sutures
What is a cystocele?
- Anterior vaginal prolapse
What is a rectocele?
- Posterior vaginal prolapse
What are some structural changes of the vagina?
- Cystocele
- Rectocele
- Uterine prolapse
- Fistulas
What is the most common cause of vaginal trauma?
- Sexual assault
What are the most common tumors of the vulva?
- Squamous cell carcinomas
Who is most likely affected by squamous cell carcinomas of the vulva?
- Postmenopausal women, age 65
What is the most frequently reported symptom of vulvar cancer?
- Long history of chronic vulvar pruritus
What is VIN usual type?
- Associated with carcinogenic HPV (type 16), smoking and immunocompromised states
- Gardasil vaccinations should cause a decrease in this type
What is differentiated type VIN?
- No associated with HPV or smoking
- More commonly associated with vulvar dermatologic conditions like lichen sclerosus
What is the management of VIN III?
- Local superficial surgical excision is mainstay of treatment
- Skinning vulvectomy which removes all vulvar skin is rarely required
- Laser therapy is useful if small lesions are on clitoris, labia minora or perianal areas
What is Paget’s disease?
- Extremely rare
- Occurs in postmenopausal white females and can occur in the nipple areas of the breast
- 10%-20% will have underlying carcinoma (breast or colon)
What are the clinical features of Paget’s disease?
- Itching and tenderness are common
- Well demarcated and eczematoid in appearance with fiery red background with white plaques like lesions
What is the management of Paget’s disease?
- Local superficial excision with 5-10 mm margins to clear the gross lesion and to exclude underlying invasive cancer
What are some clinical features of squamous cell vulvar carcinoma?
- Typically occurring in postmenopausal females between 70-80
- Vulvar lump
- Lesion is pruritic, raised, uclerated, pigmented, or warty usually on labia majora
What are the different methods of spread in squamous cell vulvar carcinoma?
- Direct extension to adjacent structures
- Lymphatic embolization to regional lymph nodes
- Hematogenous spread to distant sites
What is the management of squamous cell vulvar carcinoma?
- Radical vulvectomy and regional lymphadenectomy
- Wide local excision of the primary tumor with inguinal lymph node dissection
What is malignant melanoma of the vulva?
- Predominately in postmenopausal white women with lesions noted on the labia minora and clitoris
- Wide local excision is necessary for diagnosis and staging
What is verrucous carcinoma?
- Variant of squamous cell carcinoma
- Mets is rare
- Lesions are cauliflower-like in nature and can be confused with condyloma
- Radiation is contraindicated because it may induce anaplastic transformation
What is a bartholins gland carcinoma?
- Presents usually as a painless vulvar mass without history of previous bartholin’s gland disorders
Who usually has a bartholin’s gland carcinoma?
- Women over the age of 40 should have biopsy of gland to exclude malignancy
what is the treatment for bartholin’s gland carcinoma?
- Radical vulvectomy and bilateral lymphadenectomy with postoperative radiation
How does a basal cell carcinoma of the vulva appear? Mets??
- Rolled edge ulceration
- Does not metastasize
What is the etiology of VAIN (vaginal intraepithelial neoplasia?
- Appears to be related to the HPV viruses
- 50%-90% of patients with VAIN will have coexistent or prior neoplasia or cancer of the cervix or vulva
How is diagnosis made for VAIN?
- Asymptomatic
- Usually considered with an abnormal pap in a woman who is status post hysterectomy or has no demonstrable cervical lesion
What is the management of VAIN?
- If lesion involves the vault –> surgical excision
- Multifocal lesions –> treat with laser therapy or topical 5-fluorouracil, if unsuccessful, may require vaginectomy
What are the symptoms of carcinoma of the vagina?
- Abnormal vaginal bleeding or discharge
- Hematuria
What is the average age of someone with carcinoma of the vagina?
- 60
What are some physical exam findings of carcinoma of the vagina?
- Ulcerative
- Exophytic growth may be seen
How is diagnosis made for carcinoma of the vagina?
- Punch biopsy is required
What are some qualities of adenocarcinoma?
- Most are mets from cervix, endometrium, or ovary
- Clear cell carcinomas secondary to DES
- Treated with radical hysterectomy and vaginectomy or radiation
What are some qualities of malignant melanoma?
- Mean age is 55 and usually occurs on the distal anterior wall
- Prognosis is poor
What are some qualities of sarcoma botryoides?
- Mass of grape like polyps protruding from the introitus
- Histologically the tumor is embryonal rhabdomyosarcoma
- Average age is 2-3
What is the vagina lined with?
- Nonkeratinized stratified squamous epithelium
What are some factors that can alter the protective microflora of the vagina?
- Antibiotics
- Douching
- Intercourse (semen can raise pH or vaginal transudate has pH of 7.4)
- Foreign body
What is the most common cause of vaginitis?
- Bacterial vaginosis usually caused by Gardnerella vaginalis
What are some risk factors for bacterial vaginosis?
- New or multiple sexual partners
- Smoking
- IUD
- Douching
What are some symptoms of bacterial vaginosis?
- Many are asymptomatic
- Profuse thin milky discharge often
- Malodorous fishy amine odor especially after intercourse
How is diagnosis made for bacterial vaginosis?
- Saline wet mount reveals presence of “clue cells”
- 10% KOH positive whiff test, releases an amine like odor
- Vaginal pH >4.5
What is the most common cause of vulvovaginal candidiasis?
- Candida Albicans
What are some risk factors for vulvovaginal candidiasis?
- INcrease estrogen levels (high dose OCPs, pregnancy)
- DM, antibiotic use, steroid use, and immunosuppressed patients
What aere some symptoms of vuvlovaginal candidiasis?
- Vulvar pruritus, buringin, and irritation/dyspareunia
- Often little to no discharge. If discharge is present, it is white, adherent, and clumpy
How diagnosis made for vulvovaginal candidiasis?
- 10% KOH wet prep positive for budding yeast
- Vaginal pH <4.5
What are the risk factors for tichomoniasis?
- Unprotected sexual encounters
What are some symptoms of trichomoniasis?
- 50% of cases are asymptomatic
- Dyspareunia, vulvovaginal irritation and occasional dysuria
- Symptomatic cases reveal a green yellow “frothy” vaginal discharge
How is diagnosis made for trichomoniasis?
- Saline wet mount reveals motile trichomonads
- pH is >4.5
- Strawberry cervix
