Vulvar and Ovarian Disease Flashcards
What is lichen sclerosus?
Autoantibodies attack extracellular matrix and basement membrane (immune dysfunction affecting all levels of the skin)–poorly understood so can be genetic or enviromental too
Environmental factors affecting pathophysiology of lichen sclerosus
Incontinence
Infection
Contact dermatitis
Trauma (Kobners phenomenon)
Presentation of lichen sclerosus
Mostly in postmenopausal women
Most common sxs is pruritus!!!
Pain (dysuria and dyspareunia)
What is seen on PE for lichen sclerosus?
Sharply, well-demarcated white plaques
Usually begins periclitorally with spread to perianal skin
(not usually seen as keratinized, hair-bearing labia majora or mucus membranes)
Pathognomonic for lichen sclerosus
Plaques demonstrate “cellophane paper” (also waxy and/or hyperkeratotic apperance)
Hallmark of lichen sclerosus
Fragility (purpura, erosions and fissues)
What can occur in untreated lichen sclerosus?
Squamous cell carcinoma (small amt)
Can also see pigmentary changes (benign but can see aytpical nevi and melanoma and take the pigmented lesions seriously)
Hypothyroidism
Risk factors of developing SCC from lichen sclerosus
Elderly
Hyperkeratotic lesions!!
How to confirm diagnosis of lichen sclerosus
Vulvar punch biopsy
Tx for lichen sclerosus
Topical ultrapotent steroid ointment!!! (first line is temovate .05% ointment applied twice daily until normal texture and can use 1-3x week for maintenance)
Lifelong!!!-thicker skin so can handle the steroid for a while
Side effects of temovate steroid for lichen sclerosus
Atrophy, dermatitis and rosacea
Other tx options for lichen sclerosus
Can use topical estrogen also but does not go away
What are Bartholin ducts?
Bilateral glands at 4 and 8 o clock positions in labia minora–ducts open into vestibule adjacent to vaginal introitus–secrete mucus like material to maintain moisture of vaginal mucosa
Pathophysiology of Bartholin cyst
Cysts form as result of ductal obstruction due to trauma or non specific inflammation
Abscess formation from infected cyst or primary gland infection (polymicrobial, STIs)
Presentation of Bartholin cyst
Acute, painful unilateral labial swelling
Dyspareunia
Pain with sitting or walking
What is seen on PE for Bartholin cyst?
Tender, fluctuant labial mass Surrounding erythema and edema Cellulitis Abscess formation Fever
Tx for Bartholin cyst
I&D with insertion of Word catheter Culture purulent material Empirical abx therapy (Keflex or Doxy) Sitz baths for 2-3 days No intercourse until cath is removed
Possible pathophysiology of vulvodynia
Estrogen conc (onset around menopause, affects pain sensitivity and sensory discrimination) Pelvic floor dysfunction Psych (mood/anxiety disorders, poor allostasis) Neuro sensitization (insult to vulvar mucosa causes chronic inflammation and sensation of touch becomes painful)
Presentation of vulvodynia
Vulvar discomfort described as burning sensation (stinging, irritated, sore, raw, stabbing)
Introital pain with intercourse
Generalized vs localized (sexual or nonsexual etc)
Important parts of PE for vulvodynia
Use Q tip to palapate vestibule, labia majora, perineum or interlabial folds
Pain is limited to vestibule
Single digit exam to feel for spasm or tenderness of pelvic floor musculature
Non-specific vestibular erythema
Tx for vulvodynia
No scented products, tight clothing, vigorous exercise or pads
Sitz baths BID followed by petroleum jelly
Couple counseling
Pelvic floor PT
Local nerve block
Pharm meds for vulvodynia
Topical vaginal estrogen .03% with T .1%
Nortriptyline 50 mg QHS (titrate up starting at 10)
Gabapentin 1200 mg TID (titrate up to it)
What is vulvar intraepithelial neoplasia?
Neoplastic cells confined to squamous epithelium
How to classify vulvar intraepithelial neoplasia
1, 2 or 3 (like CIN)
Now want to combine 2 and 3 b/c true precursors to vulvar cancer (Vinu and VINd based on morphologic manifestations)
Usual type of vulvar intraepithelial neoplasia
VINu
What is VINu associated with?
HPV 16 and 18 (seen in younger women so same risk factors as CIN)
Risk factors of CIN and VINu
Smoking
Immunosuppression
Multiple sex partners
How to diagnose VINu
Vulvar colposcopy: 3-5% acetic acid and let sit for 3-5 min Avoid using acetic acid in areas of inflammation and breaks in epithelium See raised or flat lesions Color is gray to white or red to black
Presentation of VINu
Most asymptomatic
Vulvar burning and pruritus in half
Association of VINu
High grade CIN (colposcopy is mandatory to rule out)
Biopsy all pigmented lesions!!
Tx for VINu
None provide a cure (reactivate latent)
All meds are off label
Off label meds for VINu
5FU cream (many s/es)
Interferon
Imiquimod 5% cream (apply 3 times weekly up to 20 wks so low compliance)
Must do vulvar assessments q 4wks during tx
Standard of care for VINu tx
Surgery: CO2 laser vaporization (destroy entire thickness of epithelium but don't do if invasion) Local wide excision Vulvectomy Some high recurrence
What is VINd?
Differentiated type unrelated to HPV (not have same risk factors)
Seen in older women (>70)
Involves lower 1/3 of epithelium (so none abnormal cells in upper)
Pathogenesis of VINd?
Associated with squamous cell hyperplasia (lichen sclerosus, lichen simplex chronicus)
Unidentified carcinogenic agents combined with local environment of chronically irritated/inflamed skin lead to dysplastic cells
Tx for VINd
Prevent: tx of underlying
Tx is surgical excision tho
Follow up for VINu and VINd
Vaccination with Gardasil (VIN usual type)
Women with history of VIN considered at risk of recurrence
Must do post tx follow up of colposcopic vulvar inspection at 6 and 12 mos and then annually after
Incidence of vulvar cancer
Very uncommon tho
Etiology in 20-40 YO is HPV related (VINu)
60-70 YO is due to chronic irritation and poorly understood co-factors (VINd)–most have untreated lichen sclerosus, lichen simplex chronicus or squamous cell hyperplasia
Co-morbidities of vulvar cancer
Some have type 2 DM
Some are obese or hypertensive
Presentation of vulvar cancer
Asymptomatic (delays diagnosis so inspect vulva)
Pruritus is most common sx
Vulvar bleeding and pain
PE of vulvar cancer: squamous cell carcinoma
Varies in appearance from large, exophytic cauliflower like lesion to small ulcerative lesions with surrounding hyperkeratosis
PE of vulvar cancer: basal cell carcinoma
Raised lesion with ulcerated center and rolled borders
PE of vulvar cancer: malignant melanoma
Seen at labia minora and clitoris
Raised, darkly pigmented lesion
Tx for vulvar cancer
Staging based on fIGO
Primary: complete surgical removal of tumor with inguinal node dissection
Radiation therapy with lymph node spread!!
Background of vaginal intraepithelial neoplasia (VAIN)
-Precancerous disease of vagina (rare!!)
Seen mostly 35-55
HPV must be present to develop VAIN
Some have been previously treated for CIN or hysterectomy
Risk factors of vaginal intraepithelial neoplasia
Smoking, multiple sexual partners and early onset of sexual activity (same as CIN)
History of CIN III
Pathogenesis of vaginal intraepithelial neoplasia
HPV exposure but requires long time to develop
Frequency not as high as CIN since vaginal epithelium is different than cervical
Not very high progression to invasive cancer
Where are most lesions with vaginal intraepithelial neoplasia?
Upper 1/3 of vagina
Classification of vaginal intraepithelial neoplasia (VAIN)
1: benign viral proliferation
2: intermediate risk
3: true precursor to vaginal cancer
Labs for vaginal intraepithelial neoplasia
Detection is via pap smear (cytology)
Colposcopy
Management for VAIN 1
Observation when younger
Cytology/HPV/colposcopy q 6 mos
Management for VAIN 2/3
Surgical intervention or chemo
Management for vaginal intraepithelial neoplasia in general
Vaginectomy (90% success)
Laser vaporization
Topical chemo/5FU (lower success but mostly used if other options not feasible)
Most common cause of invasive vaginal cancer
Metastasis from endometrium, ovary or cervix (can only be called vaginal cancer when growth is from vagina says FIGO)
Most common type of vaginal cancer
Squamous cell
Presentation of vaginal cancer
Asymptomatic Leukorrhea Vaginal odor Post-coital bleeding Abnormal pap smear (colposcopy with acetowhite changes, punctation or mosaicism)
Tx for vaginal cancer
No standardized tx b/c rare
Combo vaginectomy and radiation (medium 5 yr survival rate)
Pathophysiology of polycystic ovarian syndrome
Abnormal androgen and estrogen metabolism
Control of androgen production is unregulated (high T, androgens, DHEA)
Insulin resistance and hyperinsulinism
Decreased adiponectin
Increased androgens released from ovary are converted to estrogen
Presentation of polycystic ovarian syndrome
Most common is infertility Oligomenorrhea/amenorrhea (anovulation) Obseity Acne and hirsutim Male pattern baldness Acanthosis nigricans
NIH criteria to diagnose polycystic ovarian syndrome
NIH criteria:
Must have oligomenorrhea and hyperandrogenism
Must exclude hyperprolactinemis, CAH and Cushings
Rotterdam criteria for polycystic ovarian syndrome
2 or 3 below must be present after exclusion of related disorders:
Oligomenorrhea
Clinical or biochemical signs of hyperandrogenism
Polycystic ovaries
(just expanded NIH)
Ultrasound findings of polycystic ovarian syndrome
Presence of >12 follicles in each ovary measuring 2-9 mm in diameter (rotterdam)
String of pearls apperance!!
Ovarian vol >10 ml
No evidence of dominant follicle/corpeus luteum
First lab for polycystic ovarian syndrome
Start by measuring total testosterone (normal is 40-60 ng/dl)
It is elevated if >60
What labs must be seen with polycystic ovarian syndrome?
Hyperandrogenism requires: 17-OH progesterone DHEA-S Cortisol Prolactin (should be normal) TSH (if increased can cause oligomenorrhea) bHCG (rule out pregnancy)
What elevated 17 OH progesterone could mean with polycystic ovarian syndrome?
Congenital adrenal hyperplasia
What elevated DHEA-S could mean with polycystic ovarian syndrome?
Adrenal source for increased testosterone
What elevated cortisol could mean with polycystic ovarian syndrome?
Cushings
Most important component of polycystic ovarian syndrome tx
Weight loss!! (increased SHBG and decrease free testosterone to restore cycling)
Other tx for polycystic ovarian syndrome
Metformin (only with hyperinsulinemia-500 BID)
COCs (low androgenic activity)—-only standard of care for these pts b/c truly helps them
Fertility consultation
Provera 10 mg QDx10 days (endometrial protection-so that they can have their normal period!)
Life long lifestyle
Risks associated with polycystic ovarian syndrome
Endometrial hyperplasia/carcinoma Type 2 DM HTN HLD CVD Stroke Infertility Metabolic syndrome Sleep apnea
Ultrasound characteristics of benign adnexal mass
Thin walled
<3 cm premenopause of <1 cm post menopause (simple cyst)
Hyperechoic nodule with distal acoustic shadowing (teratoma)
Network of linear or curvilinear pattern (hemorrhagic cyst)
Homogenous echos (endometrioma)
Ultrasound characteristics of malignant adnexal mass
Thick septations >2 mm
Solid component appears nodular or papillary
Blood flow to solid component
Types of physiologic ovarian cysts
Follicular cysts Corpeus luteum cysts Theca lutein cysts Mature teratoma Serous and mucinous cystadenomas
Most common type of ovarian cyst
Follicular
Characteristics of follicular ovarian cystq
2-8 cm
Non malignant
Results from failure or mature follicle to rupture (release ovum) or failure of non-dominant follicles to undergo atresia in presence of mature follicle
Characteristics of corpus luteum ovarian cyst
3-11 cm
Less common
Following ovulation, blood accumulates in cavity of corpus luteum which stimulates resorption (if that doesnt occur and the luteum is greater than 3 cm it is a cyst)
When do corpus luteum cysts usually resolve?
After 1-2 menstrual cycles
Characteristics of theca lutein cysts
Seen with elevated chorionic gonadotropin levels (hydatidiform mole, choriocarcinoma, clomid therapy)
Usually bilaterally
Fluid is clear or straw colored
-Usually due to stimulation (fertility tx!!)
When do theca lutein cysts resolve?
Spontaneously with tx of underlying disorder
What are mature teratomas?
Half of benign neoplasms in reproductive age women
Pathophysiology of mature teratoma
Parthenogenic theory: originate from primordial gem cells and teratomas found along migration pathway of germ cells from yolk sac to gonads)
Composed of well differentiated tissue derived from any of 3 derm layers (ectoderm, mesoderm, endoderm)
Histology of mature teratoma
Cyst is lined with keratinized squamous epithelium with abundant sebaceous and apocrine glands
(can have hair and teeth!!!)
Most common origin of mature teratoma
Ectodermal cell origin
Presentation of mature teratoma
Asymptomatic (found on pelvic exam or incidental finding on other radiologic studies)
Pelvic pain (occurs secondary to torsion or rupture)
Frequency or urgency
Back pain
What is seen on PE for mature teratoma?
Pelvic mass on bimanual exam
Labs for mature teratoma
Transvaginal u/s (unilateral, complex xyst)
CEA, CA-125, AFP, bHCG (all should be normal b/c signs of ovarian cancer!)
Tx for mature teratoma
Laparotomy vs laparoscopy
Ovarian cystectomy vs oophorectomy
Recurrence is low
What is the background of ovarian cyst serous/mucinous cystadenoma?
30-50 YO
Represents 20-25% of benign neoplasm
Histology of ovarian cyst serous/mucinous cystadenoma
Lined with columnar epithelium
Secrete think, gelatinous mucin (mucinous)
Thin walled, uni or multilocar and range in size from 5 to over 20 cm (mucinous greater than serous)
Tx for ovarian cyst serous/mucinous cystadenoma
Surgical excision and ensure benign pathology
2nd most common gynecologic cancer
Ovarian cancer
Most common cause of gynecologic cancer death in US
Ovarian cancer
Risk factors of ovarian cancer
Nulliparity!!, infertility tx, diets high in saturated animal fats, obese, talcum powder, personal Hx of breast cancer, family hx of breast, OVARIAN or colorectal cancer (higher with BRCA1 or lynch), Turners, early menarche!! or late menopause!!, estrogen replacement therapy, caucasian, endometriosis
***more women ovulates, more at risk she is
Ways to reduce the risk of ovarian cancer
Multiparity Breast feeding Long term oral contraceptive use (5 yrs drops it by 50%) Bilateral tubal ligation Low fat diet Bilateral salpingectomy!!!!
4 categories of ovarian cancer on histopathology
Epithelial (bilateral and older)
Germ cell (unilateral and younger)
Sex cord and stromal
Neoplasms metastatic to ovary
Subtypes of epithelial ovarian cancer
High grade serous carcinoma
Endometriod carcinoma
Clear cell carcinoma
Mucinous carcinoma
Subtypes of germ cell ovarian cancer
Dysgerminoma Endodermal sinus Immature teratoma Embryonal carcinoma Choriocarcinoma
Subtypes of sex cord and stromal ovarian cancer
Granulosa cell
Sertoli-stromal cell
Examples of neoplastims metastatic to ovary
Tumors from stomach, colon or breast
Theory of epithelial cancer based on location in ovary
Incessant ovulation therapy (repeated ovarian epithelital trauma by follicular rupture and subsequent epithelial repair and leads to malignant transformation)
Associated with endometriod, mucinous or clear cell cancer in ovary
Theory of epithelial cancer based on location in fallopian tube
p53 tumor suppressor gene
Associated with high grade serous papillary cancer!!
Types of ovarian cancer epithelial neoplasms
High grade serous carcinoma MOSTLY (from fallopian tube- why salpingectomy decreases risk) Endometriod carcinoma (from ovary) Clear cell carcinoma (from ovary) Mucinous carcinoma (from ovary)
Background of germ cell ovarian cancer
20-30 YO mostly
Grow rapidly, favor lymphatic spread, contain mixture of tumor types and usually unilateral
They produce tumor markers
-*arises from internal party of ovary (epithelial is from outside)
Types of germ cell ovarian cancer
Dysgerminoma Endodermal sinus tumor Immature teratoma Embryonal carcinoma Choriocarcinoma (mixed can be combo of 1-3)
Characteristics of germ cell ovarian cancer: dysgerminoma
Most common!!
Unilateral mostly
Mostl < 30 YO
Characteristics of germ cell ovarian cancer: endodermal sinus tumor
Bilateral
Displays most rapid growth of germ cell neoplasms
Produces alpha fetoprotein
Characteristics of germ cell ovarian cancer: immature teratoma
2nd most common
Seen mostly <20 YO
Unilateral mostly
Produces alpha fetoprotein
Characteristics of germ cell ovarian cancer: embryonal carcinoma
Uncommon
Rapid growth with extensive
Produces alpha fetoprotein and HCG
Characteristics of germ cell ovarian cancer: choriocarcinoma
Seen with precocious puberty, uterine bleeding or amenorrhea
Very rare
Mostly 2nd decade of life
Types of sex-cord stromal tumors associated with ovarian cancer
Granulosa cell
Sertoli-Stromal cell
Characteristics of sex-cord stromal tumors associated with ovarian cancer: granulosa cell
Most common!
Causes hyperestrogenism (precocious puberty and post menopausal bleeding)
5th decade of life
Characteristics of sex-cord stromal tumors associated with ovarian cancer: sertolid-stromal cell
Rare
Causes hyperandrogenism
3-4th decades
Presentation of ovarian cancer
Acute: pleural effusion and bowel obstruction
Subacute sxs: adnexal mass, bloating/abd distention, early satiety, pelvic/abd pain, abnormal vaginal bleeding, altered BMs, dyspepsia
What is seen on PE for ovarian cancer?
Ascites
Inguinal LAD
Pelvic mass
Imaging for ovarian cancer
Transabd/vaginal u/s (BEST-verify mass)
Mammogram/colonoscopy rule out primary breast or colorectal cancer)
CT (reveal retroperitoneal involvment and metastais)
MRI (characteristics of neoplasms)
CXRs
Labs for ovarian cancer
CA-125 elevated (suspected epitheltial ovarian cancer!!! >65 U/mL)
Elevated hCH, AFP, LDH or any variation of these 3 (suspected germ cell tumor!!!)
**order all four with an adnexal mass where suspicious for ovarian cancer
Tx for epithelial ovarian cancer
Gyn ocology refer!!
Surgical staging (FIGO)
Chemo
Tx for germ cell ovarian cancer
Consult gyn oncologist
Early diagnosis allows removal of involved adnexa with preservation of contralateral adnexa and uterus
Surgical staging (FIGO
