Benign and Malignant Conditions of the Vulva and Vagina Flashcards
Ambiguous genitalia can present with:
1.
2.
-which results secondary to
3.
4.
5.
-which is:
- clitoromegaly
- clitoral agenesis
- which results secondary to failure of the genital tubercle to form
- bifid clitoris
- midline fusion of the labiascrotal folds
- cloaca
- which is: no definite separation between the vagina and bladder
Female pseudohermaphroditism
Is caused by:
Due to________________ such as:
1)
2)
3)
The most obvious abnormalities are:
1)
2)
3)
_________ is normal
Caused by: masculinization in utero of the female fetus
Due to: endogenous hormonal milieu (environment/setting)
Such as:
- Congenital adrenal hyperplasia
- Ingestion of exogenous hormones
- Androgen secreting tumors of the mother’s adrenal or ovaries
The most obvious abnormalities are:
- clitoromegaly
- hypospadiac urethra meatus
- malpositioned vaginal orifice
internal genital organ development is normal
Male Pseudohermaphroditism
Commonly results from:
Can occur with varying degrees of:
-i.e. _________________ (_____________)
Commonly results from: mosaicism
Can occur with varying degrees of: virulization and mullerian development
-i.e. androgen insensitivity syndrome (Testicular feminization)
Androgen Insensitivity
Caused by:
Karyotype:
Inheritance pattern:
Results in:
Presentation:
__________ is produced by the 46 XY which results in: ______ (_____)
___________ can be seen in partial androgen insensitivity
Caused by: genetic deficiency in androgen receptors
Karyotype: XY
Inheritance pattern: 46 XY x-linked recessive
Results in: external female phenotypic development
Presentation: testes are undescended (inguinal canal or labia)
Mullerian inhibiting substance is produced by the 46 XY which results in lack of mullerian duct development (absent uterus or fallopian tubes)
Ambiguous genitalia can be seen in partial androgen insensitivity
Androgen insensitivity syndrome (46,XY) is most commonly inherited how?
X-linked recessive
Structural and Benign Neoplastic Conditions of the Vuvla
How do you treat labial agglutination?
Treated by estrogen cream and massage to separate the labia majora
Structural and Benign Neoplastic Conditions of the Vulva
What is Fox-Fordyce disease?
Severe pruritic raised yellow retention cyst in the axilla and labia majora and minora resulting from keratin-plugged inflammation of apocrine glands
Structural and Benign Neoplastic Conditions of the Vulva
Where are inclusion cysts located?
How do they present?
beneath the epidermis
- they are mobile, nontender, spherical, and slow growing
- Develop when the hair follicles become obstructed; the deeper portion of the follicle swells to accommodate the desquamated cells
What is the most common type of genital cyst?
epidermal inclusion cyst
Structural and Benign Neoplastic Conditions of the Vulva
How do urethral caruncles appear?
-Appear as a small fleshy red outgrowth at the distal edge of the urethra
Urethral caruncles in children are caused by what?
Urethral caruncles in post menopausal women are secondary to what?
Children: caused by the spontaneous prolapse of the urethral epithelium
Post menopausal women: secondary to contraction of the hypoestrogenic vaginal epithelium resulting in everting of the urethral epithelium
What is the most common type of genital cyst?
Epidermal inclusion cyst = mobile, nontender, spherical, and slow growing
What is vulvar vestibulitis?
How are the lesions characterized?
How is it treated?
Rare condition in which one or more of the minor vestibular glands becomes infected
Lesions are 1-4 mm erythematous dots that are extremely tender; characterized by severe introital dysparunia and occasionally vulvar pain
Can try topical estrogens/hydrocortisone or surgical therapy
Sebaceous cysts of the vulva are most commonly found where and contain what?
- Inner surface of labia minora and majora
- Contain a cheesy sebaceous material
What is the most common benign solid tumor of the vulva; what are its growth characteristics?
- Fibromas
- Slow growing, most range from 1-10cm
- CAN become gigantic (250 lbs!!!!)
What is a hidradenoma?
a rare lesion arising from sweat gland of the vulva
What is syringoma?
Eccrine gland tumor
How do angiomas of the vulva appear?
as multiple 2-3 mm red lesions usually in the 4th and 5th decade
Which condition of the vulva is assoc. w/ 1-4mm erythematous dots that are extremely tender and is characterized by severe introital dyspareunia and occasional vulvar pain?
Vulvar vestibulitis (vestibular adenitis)
What is the treatment for labial agglutination?
Estrogen cream and massagetoseparatethelabia majora
What are vulvar hematomas?
Vuvlvar hematomas most often arise following what; how are they managed?
- Loculated collections of blood that collect
- Arise following trauma i.e., bike injuries (straddle injury), birth trauma or sexual assault
- Close observation and occasional surgical exploration may be warranted
What are the different classifications of female genital mutilation?
Type I: partial removal of the clitoris and/or prepuce
Type II: Partial or total removal of the clitoris and labia minora, with or without excision of the labial majora
Type III: Narrowing of the vaginal orifice with creation of a covering seal by cutting and appositioning the minora and/or majora with or without excision of the clitoris
Type IV: all other harmful procedures to the female genitalia for nonmedical purposes (pricking, piercing, incising, cautery)
Atrophic vaginitis is due to what?
What does exam reveal?
managed how?
- Due to loss of estrogen (seen in menopause or after surgery)
- Exam reveals atrophy of external genitalia: minora regresses and majora shrinks; loss of vaginal rugae; vaginal introitus constriction
- Tx w/ topical estrogen and may consider oral estrogen to prevent recurrence
What is Lichen simplex chronicus?
What are the symptoms?
Exam reveals?
Biopsy reveals?
- Squamous cell hyperplasia
- Local thickening of epithelium that results from a prolonged itch-scratch cycle
- symptoms include pruritus
- Exam reveals white or reddish thickened, leathery, raised surface; looks similar to psoriasis
- Biopsy reveals elongated rete ridges and hyperkeratosis of the keratin layer
Treatment for lichen simplex chronicus?
Moderate strength steroid ointments w/ anti-pruritic agents
Where is lichen sclerosis most frequently found?
What can it cause?
What will some women with lichen sclerosis later develop?
What are the symptoms of lichen sclerosis?
Examination reveals?
on the vulva of menopausal women
Can cause genital structural abnormalities
4% of treated women and 10% of untreated women will develop squamous cell cancer of the vulva
intense pruritus, dyspareunia, and burning pain
Examination reveals thin, white, inelastic skin with a crinkled tissue paper appearance “onion skin, cigarette paper, parchment like”
Biopsy of lichen sclerosis will show what 2 major characteristics?
- THIN epithelium
- Loss of rete ridges and inflammatory cells lining the BM
Treatment for lichen sclerosis?
clobetasol 0.05%
What is the most striking feature of lichen sclerosis?
the presence of a hyaline zone in the superficial dermis
What is seen with lichen planus and what are the sx’s?
- Purplish, polygonal papules that may appear in an erosive form
- Sx’s: vulvar burning, severe insertional dyspareunia
- Tx: topical and systemic steroids
What is vulvar-vaginal-gingival syndrome?
when lichen planus involves the vulva, vagina, and mouth
What is psoriasis and how does it appear on the vulva?
autosomal dominant inherited disorder
on the vulva it generally appears velvety but may lack the silver scaly patches found on flexor surfaces
What is pemphigus?
autoimmune blistering disease involving the vulvovaginal and conjunctival areas
What is bechet’s syndrome?
classically involves ulcerations in the genital, oral areas with uveitis
How does Crohn disease affect the vulva?
it is primarily a GI disorder but vulvar ulcerations can occur due to fistulizations
What are the different types of vaginal septum that can form?
transverse
Midline longitudinal
Where 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
What does a midline longitudinal septum create?
What are these septa usually associated with?
- Creates a double vagina, a longitudinal septum can attach to the lateral wall thus creating a blind vaginal pouch
- These septa are usually associated with various duplication anomalies of the uterine fundus
In cases of vaginal agenesis, there is total absence of the vagina except for what portion?
the most distal portion, which is derived from the urogenital sinus
What is Rokintansky-Kuster-Hauser syndrome?
Vaginal agenesis, if the uterus is absent but the fallopian tubes are spared
-due to mullerian agenesis
What is adenosis?
In what population of women is it seen in?
congenital anomaly of 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 do Gartner’s duct cyst arise from?
How do they present?
Arise from the remnant of the Wolffian duct (mesonephros)
- 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, .3-3.0 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
Structural and Benign Neoplastic Conditions of the Vagina
Inclusion Cysts
Result from?
Located?
Associated with?
Result from 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 childbirth
What is the most common vulvovaginal tumor?
How do they present?
Bartholin’s cyst
Less than 3cm is usually asymptomatic
Usually unilateral swelling
Need to biopsy in women 40+ years to rule out Bartholin’s carcinoma
What is bartholin’s gland abscess?
results from blockage and accumulation of purulent material
painful inflammatory mass arises
How do you treat bartholin’s gland abscess?
- Word catheterization
- Marsupialization: creates a new ductal opening by everting the cyst wall onto the epithelial surface where it is sutured with interrupted absorbable sutures
What are 4 examples of structural changes of the vagina that can occur over time?
cystocele: anterior vaginal prolapse
Rectocele: posterior vaginal prolapse
Uterine prolapse
Fistulas: can result from radiation, obstetric injuries, complications of surgery
What is dermatologic atrophy of the vagina?
after menopause the vaginal rugations flatten out and the vaginal epithelium become thin, pale, and inelastic
-vaginal pH rises
Most vulvar neoplasms are what type?
Most occur in what population of women?
most are squamous cell carcinomas
occur mainly in postmenopausal women
most frequently reported symptom of vulvar cancer is long history of chronic vulvar pruritus
___________ aka squamous cell carcinoma in situ has been linked to vulvar cancer
-lesions are designated ________ based upon _______
Vulvar intraepithelial III (VIN III) aka squamous cell carcinoma in situ has been linked to vulvar cancer
-lesions are designated VIN I, VIN II, and VIN III based upon the depth of epithelial involvement
Vulvar intraepithelial neoplasia (VIN) type III is used to denote what?
and i subdivided into two types: what are the two types?
used to denote high grade squamous lesions
subdivided into two types:
VIN Usual type
Differentiated type VIN
What is the VIN usual-type VIN type III associated with?
associated with carcinogenic HPV (type 16), smoking, and immunocompromised status
*Gardasil vaccinations should cause a decrease in this type
What is the differentiated type VIN associated with?
more commonly associated with vulvar dermatologic conditions, such as Lichen sclerosis
What are the clinical features of VIN III?
pruritus is the most common symptom
there is no absolute diagnostic appearance
20% of the lesions have a warty appearance
What is the management of vulvar intraepithelial neoplasia type III?
local superficial surgical excision is mainstay of treatment
*disease seldom goes beyond lesion, so 5 mm margins are usually adequate
*laser therapy is useful if small lesions are on clitoris, labia minora, or perianal areas
What is preinvasive disease of the vulva- PAGET’S Disease?
- extremely rare
- occurs in postmenopausal white females and can also occur in the nipple areas of the breast
10-20% of patient’s with vulvar pagets disease will have an underlying carcinoma (breast or colon)
Clinical features:
itching and tenderness
well demarcated and eczematoid in appearance with fiery red background with white plaque like lesions
Histologic features: biopsy reveals large pale pathognomonic Paget’s cells
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 the clinical features of squamous cell vulvar carcinoma?
typically occurring in post menopausal females between 70-80 y/o
vulvar lump
present with a lesion that is pruritic, raised, ulcerated, pigmented or warty in appearance usually on labia majora
definitive diagnosis requires a biopsy
What are the methods of spread of squamous cell vulvar carcinoma?
direct extension to adjacent structures (vagina, urethra, and anus)
lymphatic embolization to regional lymph nodes
hematogenous spread to distant sites (lung, liver, bone)
What is the characteristic age range of those affected with Type I (usual type) VIN III?
35-65 years old (younger)
what is the characteristic age range of those affected with type 2 (differentiated VIN) VIN III?
55-85 years old (older)
What is the management of squamous cell vulvar carcinoma?
radical vulvectomy and regional lymphadenectomy or
wide local excision of the primary tumor with inguinal lymph node dissection +/- preoperative radiation, chemotherapy, or both
*stage I rarely has positive contralateral nodes and thus ipsilateral lymphadenectomy is sufficient
*if positive nodes are identified post op radiation is needed
What is the second most common vulvar cancer?
who does it predominantly occur in?
malignant melanoma
predominantly occurs in postmenopausal white women with lesions noted on the labia minora and clitoris
What is verrucous carcinoma?
a variant of squamous cell carcinoma
metastasis is rare
lesions are cauliflower-like in nature which can be confused with condyloma
***radiation is contraindicated because it may induce anaplastic transformation
What is bartholins gland carcinoma?
accounts for 1-2% of vulvar carcinomas
presents usually as a painless vulvar mass without history of previous bartholin’s gland disorders
women over the age of 40 should have biopsy of gland to exclude malignancy
treatment is radical vulvectomy and bilateral lymphadenectomy with postoperative radiation
recurrence is common
What is vaginal intraepithelial neoplasia (VAIN)?
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
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 vaginal intraepithelial neoplasia (VAIN)?
if lesion involves the vault: surgical excision is indicated to treat VAIN and exclude vaginal cancer
Multifocal lesions: treat with laser therapy or topical 5-fluorouracil if unsuccessful may require vaginectomy
what are the symptoms of carcinoma of the vagina?
physical exam findings
abnormal vaginal discharge or bleeding; hematuria
Physical exam: ulcerative, exophytic growth
Patterns of spread of carcinoma of the vagina?
direct spread into the bladder, urethra, rectum or lateral side wall
lymphatic spread
hematogenous spread
how do you make the diagnosis of carcinoma of the vagina?
punch biopsy is required to confirm diagnosis
Most primary vaginal adenocarcinomas are metastatic usually from?
the cervix, endometrium, or ovary
What is sarcoma botryoides?
presents as a mass of grape-like polyps protruding from the introitus
histologically the tumor is embryonal rhabdomyosarcoma
mean age is 2-3 years old
treatment consists of surgical resection, chemotherapy, +/- radiation
what type of epithelium lines the vagina?
nonkeratinized stratified squamous epithelium
what organisms predominate the vagina?
lactic acid and hydrogen peroxide producing lactobacilli which results in keeping the vaginal pH 3.8-4.2
what instrument is used to determine vaginal pH?
nitrazine paper
what is the most common cause of vaginitis?
describe this condition
risk factors:
symptoms:
diagnosis:
treatment:
bacterial vaginosis
often polymicrobial but Gardnerella vaginalis is one of the most common organisms present in BV
risk factors: new or multiple sexual partners, smoking, IUD, and douching
symptoms: many patients may be asymptomatic, profuse thin milky discharge often; malodorous fishy amine odor especially after intercourse
diagnosis: saline wet mount reveals presence of “clue cells”; 10% KOH-positive whiff test, releases an amine-like odor; vaginal fluid pH >4.5
treatment: metronidazole 500 mg BID x7 days
what are clue cells?
epithelial cells covered with bacteria
What is the second most common cause of vaginal infections?
most common cause:
risk factors:
symptoms:
diagnosis:
treatment:
vulvovaginal candidiasis
most common cause: candida albicans
risk factors: increase estrogen levels (high dose OCPs, pregnancy); DM, antibiotic use, steroid use, and in immunosuppressed patients
symptoms: vulvar pruritus, burning, and irritation/dyspareuina; often little to no discharge. if discharge present it is white adherent and clumpy (cottage cheese like)
diagnosis: 10% KOH wet prep- positive for budding yeast; vaginal pH is <4.5
treatment: diflucan 150 mg x1; vaginal application with synthetic imidazoles
What causes trichomoniasis?
risk factors:
symptoms:
diagnosis:
treatment:
caused by the flagellated protozoan T. Vaginalis
risk factors: unprotected sexual encounters
symptoms: 50% of cases are asymptomatic; dyspareunia, vulvovaginal irritation, and occasional dysuria; symptomatic cases reveal a green-yellow “frothy” vaginal discharge
diagnosis: saline wet mount reveals motile trichomonads; pH is> 4.5; strawberry cervix
treatment: metronidazole 2 grams single dose; IS a sexually transmitted disease, test patient for STIs and have partners be evaluated and tested for STI
Transverse vaginal septums are most commonly found where in the vagina and may only become apparent when?
- Upper and middle thirds of the vagina
- May only become apparent when intercourse is impeded
Mullerian agenesis characterizd by absence of the uterus but sparing of the fallopian tubes is known as what?
Rokintansky-Kuster-Hauser Syndrome
What is the most common vulvovaginal tumor?
Bartholin’s Cyst
Bartholin’s cysts are typically (uni-/bilateral); how does size dictate symptoms and when must you biopsy?
- Typically unilateral swelling
- <3cm is usually asymptomatic
- Need to biopsy in women 40+ y/o to rule out a Bartholin’s carcinoma!
What are 2 treatment options for bartholin’s gland abscess?
- Word catheterization: left in for 4-6 wks which promotes an epithelialized tract for drainage of glandular secretions
- Marsupialization: creates a new duct opening by everting the cyst wall onto the epithelial surface where it is sutured w/ interrupted absorbable sutures
What is the most frequently reported symptom of vuvlar cancer?
Long history of chronic vulvar pruritus
What is VIN III usual-type vs. differentiated-type?
- Usual-type: assoc w/ HPV (16/18), smoking, and immunocompromised states; younger (35-65 y/o)
- Differentiated-type: is NOT assoc. w/ HPV or smoking –> more commonly w/ vuvlar dermatologic conditions, such as Lichen Sclerosus; older (55-85 y/o)
Paget’s disease of the vuvla is most common in whom and what are the signs/sx’s?
- Postmenopausal white females
- Itching and tenderness are common —> well-demarcated and eczematoid in appearance w/ fiery red background w/ white plaque-like lesions
How is VIN type III managed clinically?
- Local superficial surgical excision is mainstay of tx; 5mm margins are typically adequate
- Can do skinning vulvectomy
- Laser therapy is useful for small lesions on clit, labia minora or perianal areas
What is the management for SCC of the vulva?
- Radical vulvectomy and regional lymphadenectomy
or
- Wide local excision of the 1’ tumor w/ inguinal LN dissection +/- pre-op radiation, chemo, or both
What do the lesions of verrucous carcinoma of the vuvla look like; what kind of tx is contraindicated?
- Lesions are cauliflower-like and can be confused w/ condyloma
- Radiation = contraindicated because it may induce anaplastic transformation
What is tx for Batholin’s gland carcinoma?
Radical vulvectomy and bilateral lymphadenectomy w/ post-op radiation
When is the diagnosis of vaginal intraepithelial neoplasia (VAIN) usually considered?
When an abnormal pap in a woman who is status post-hysterectomy or has no demonstrable cervical lesion
What is the main method of treatment for carcinoma of the vagina?
Radiation or chemoradiation
When collecting sample for investigation of vaginal discharge where do you take the sample from?
Sample discharge from posterior fornix and place on slide
Diagnosis of Gardnerella vaginalis as cause of vaginitis can be made with what 3 findings?
- Saline wet mount reveals presence of “clue cells”
- 10% KOH-positive whiff test
- Vaginal pH >4.5
What is treatment for Gardnerella vaginalis?
Metronidazole BID x 7 days
Sx’s and characteristics of the discharge assoc. w/ vuvlovaginal candidiasis?
- Vulvar itching, burning, irritation/dyspareunia
- Often little to no discharge, but if present is white adherent and clumpy (cottage cheese-like)
Diagnosis of vuvlovaginal candidiasis made via what 2 findings?
- 10% KOH wet prep-positive for budding yeast
- Vaginal pH <4.5
Treatment for vulvovaginal candidiasis?
- Diflucan
- Vaginal application w/ synthetic imidazoles (miconazole, terconazole, etc.)
What is the vaginal discharge like w/ symptomatic T. vaginalis?
Green-yellow “frothy” discharge
How is diagnosis of T. vaginalis made?
- Saline wet mount reveals motile trichomonads
- pH >4.5
- Strawberry cervix
What is the treatment for T. vaginalis; must also evaluate for what?
- Metronidazole
- Is an STD, so test pt for STI’s and have partners evaluated too!
