Pathology of the Vulvar/Vagina

Question 1

Persistent/progressive inflammatory dermatosis of unknown etiology

Answer 1

Lichen sclerosus

AKA Chronic atrophic vulvitis AKA Lichen sclerosus et atrophicus

with predilection for vulva; may be perianal Very thin, white, itchy skin

Usually age 40 years or older (more common after menopause)

Rare in children, may involute spontaneously at puberty

May be familial; associated with autoimmune diseases

Question 2

Lichen sclerosus:

1. Associated risk with what cancers?

Answer 2

Benighn:

2. NOT A PREMILIGNANT! But ass. with

VIN > squamous cell carcinoma 9% developed VIN, 21% developed invasive squamous cell carcinoma (mean 4 years later)

Question 3

Non-specific condition resulting from rubbing or scratching to relieve pruritis

1. underlying causes?

Answer 3

Lichen simplex chronicus AKA squamous cell hyperplasia

include specific infection (e.g. candida) and irritants.

May be idiopathic Generally considered a benign (not premalignant) lesion, but sometimes seen near areas of with squamous cell carcinoma

Question 4

Gross features of Lichen simplex chronicus

Answer 4

Gross:

thick, scaly plaques with erythema, well demarcated from surrounding skin;

associated with excoriation, lichenification

Micro:

irregular acanthosis, orthokeratosis and parakeratosis; dermal fibrosis, hyperplasia of small dermal nerve trunks within dermal scars TX: topical steroid or topical calcineurin inhibitor

Question 5

vaginitis can be: What are the pH ranges of these disorders?

1.

2.

3.

Answer 5

1. bacterial vaginosis: pH>4.5
2. vulvovaginal candidiasis: <= 4.5 (normal)
3. trichomonas >4,5

Question 6

characteristics of the vaginal discharge that should be noted during examination:

1.

2.

3.

4.

Answer 6

Color

Viscosity

Adherence to vaginal walls

Presence of an odor.

Question 7

A discharge specimen should be collected from the ____ of the vagina

Answer 7

lateral wall

Question 8

Diagnosis of VD can be concluded by what lab findings?

1. pH
2. Special cells
3. Special test. What chemical is used?
4. Discharge

Answer 8

1. pH >4.5, which is most sensitive but least specific sign.
2. The presence of 20% clue cells (bacterial clumping upon the borders of epithelial cells) on wet mount examination.
3. Positive amine, “whiff” or “fishy odor” test (liberation of biologic amines with or without the addition of 10% KOH). 4. Homogeneous, nonviscous, milky-white discharge adherent to the vaginal walls.

Question 9

Bacterial clumping upon the borders of epithelial cells are called __. This indicates what infection?

Answer 9

Clue cells

Bacterial Vaginosis

Question 10

Homogeneous, nonviscous, milky-white discharge adherent to the vaginal walls

Answer 10

BV

Question 11

Gold standard for BV diagnosis

Answer 11

Gram Stain

Question 12

What does a normal Gram stain of vaginal discharge show?

Answer 12

1. lactobacillus (long Gram-positive rods) only or lactobacillus with few Gardnerella morphotypes.

Question 13

What findings are indicative of BV on a smear (wet mount)?

Answer 13

When a more mixed flora is present and lactobacillus is absent or present in low numbers

Will see granular appearance and shaggy looking cytoplasm from clue cells

Question 14

Embyrological developement of vagina:

upper 2/3

Lower 1/3

Answer 14

1. paramesonephric ducts AKA Mullerian ducts
2. lower third of vagina derived from urogenital sinus.

Question 15

Findings that indicate Trichomonas

1. Wet mount
2. pH

Answer 15

1. Motile trichomonads seen in a saline wet mount (most common method)! This is required!
2. pH>4,5
3. NAAT (nulceic acid amp test)

Question 16

Strawberry cervix

Answer 16

Trichomonas

Question 17

Sensitivity, specitivity of the following tests in order

culture vs OSOM vs. wet mount vs. Affirm

Answer 17

culture> OSOM/Affirm> wet mount

Question 18

Presence of any persistent Mullerian type columnar glandular epithelium in vagina

Answer 18

Adenosis

Question 19

Explain the etilogy of Adenosis

Answer 19

•Normally during development stratified squamous epithelium in the lower third of the vagina that is derived from the urogenital sinus migrates upward and replaces the columnar type epithelium in the upper 2/3 of the vagina that was derived from Mullerian ducts

Question 20

Diethylstilbestrol (DES): Risks of :

woman that took is

Duaghters

Sons

Answer 20

•Women that took DES

–Increased risk of breast cancer

•Daughters of mothers that took DES during pregnancy

1. vaginal adenosis
2. clear cell adenocarcinoma
3. abnormalities in formation of uterus and fallopian tubes

•Sons of mothers that took DES during pregnancy

1. increased risk of epididymal cysts
2. cryptorchidism
3. testicular inflammation/infection

Question 21

Clear cell adenocarcinoma

1. Is this malignant?
2. Mean age of pt?
3. Increased risk in pt with ___

Answer 21

1. yes
2. Mean age 22 years
3. vaginal adenosis 2/3 have history of in utero DES exposure

Question 22

A biopsy reveals malignant cells that have abundant clear cytoplasm (glycogen) arranged in a mixture of glandular, cystic, solid and papillary patterns, glands often contain hobnail cells.

Answer 22

Clear cell carcinoma

Question 23

Hobnail cells are found in what diseases? Describe morphological appearance.

1.

2.

3.

Answer 23

1. clear cell ovarian adenocarcinoma,
2. collecting duct carcinoma
3. end-stage cirrhosis.

Question 24

A 2 year old girl presents to the ED with •vaginal a grape-like mass projects out of the vagina.

1. What microscopic features would you expect to find?
2. What markers would be positive on staining?
3. What is th dx and tx?

Answer 24

–1. spindle-shaped tumor cells with cross-striations in cytoplasm (rhabdomyoblasts) as well as cambium layer: tumor cells crowded beneath intact vaginal epithelium

2. Stain positively for desmin, actin, and myogenin

Embryonal Rhabdomyosarcoma

Tx: surgery combined with multidrug chemotherapy

Question 25

A woman is diagnosed with Vaginal squamous cell carcinoma. 1. Where does this tumor arise? 2. What predisposing factors can cause this? 3. What is a precursor fnding that can identify the risk of developing this cancer?

Answer 25

1. Malignant neoplasm arising in vaginal squamous epithelium 2. HPV 3. Get dysplasia (vaginal intraepithelial neoplasia) initially as precursor to carcinoma

Question 26

A woman is dx with Vaginal squamous cell carcinoma. Where are metastasis most likely to occur in the tiumor is found in the lower 3rd of the vagina vs. the upper 2/3?

Answer 26

lower third of the vagina metastasize to the inguinal nodes upper two-thirds of the vagina tend to spread to regional iliac nodes

Question 27

Portion of the female genitalia that consists of the **skin and mucosa external to the hymen** What type of epithelial tissue is found here?

Answer 27

Vulva ## Footnote •Squamous epithelium

Question 28

Lichen Sclerosis features: Gross Microscopic

Answer 28

Gross: atrophic skin resembles cigarette paper (crinkly atrophy); often with loss of labia; also white/red ill-defined patches Micro: : hyperkeratosis; thin epidermis, loss of rete pegs, homogenized band of dense fibrosis at **papillary dermis**, chronic inflammation around vessels that is often band like TX: Topical steroids, calcipotriol cream, topical and systemic retinoids (acitretin), and systemic steroids.

Question 29

parakeratosis vs

Answer 29

Hyperkeratosis with retention of nuclei in stratum corneum ass. with psoriasis

Question 30

What is VIN? What changes do you see if associated with HPV?

Answer 30

Vulvar Intraepithelial Neoplasia involves dysplastic changes to normal vaginal cells. Doesn't always lead to cancer Koilocytic change (cells with perinuclear halos)

Question 31

VIN (Vulvar Intraepithelial Neoplasia) is dz in a 60 year old woman. The woman also has a PH for lupis. What findings would indicate whether she has classic or differentiated VIN?

Answer 31

If Differentiated: •Atypia confined to lower layers of epithelium only (in the basal and parabasal layers) P53 mutation Lichen: sclerosis or simplex chronicus NO HPV!!! If classic then HPV is prevelant •discrete white (hyperkeratotic) or a slightly raised, pigmented lesion.

Question 32

VIN treatment and care

Answer 32

•VIN can be treated with excision, laser ablation, or topical imiquimod (off-label use). Monitor for vulvar cancer every 6-12 months annualy

Question 33

Vulvar Squamous Cell Carcinoma 2 groups: 1. 2.

Answer 33

•Two main groups 1. Keratinizing squamous cell carcinomas (65-80% of cases) * unrelated to HPV infection. * older women. 2. Basaloid and warty carcinomas (20-35% of cases) related to infection with high risk HPV types, most commonly HPV-16. * These occur at younger age.

Question 35

paired structures which lie deep to the posterior ends of the labia minora

Answer 35

Bartholin’s Glands duct on each side opens between the labium minus and the hymen and is about 0.5 cm long * Secrete lubricating mucus * Normally the gland cannot be palpated

Question 36

Bartholin’s Gland Cysts 1. often related to what infection? Treatment:

Answer 36

Gonnorrhea ## Footnote •: Word catheter, surgery, marsupialization, excision, antibiotics for infection

Question 37

This •is usually a primary malignancy involving the labia majora and is a glandular tumor of vulva. If secondary, what dx are related? 1. 2.

Answer 37

Extramammary Paget’s Disease 15-30%: 1. adenocarcinoma of anorectum 2. urothelium, prostate)

Question 38

Extramammary Paget’s Disease: What other cancer must this be differentiated from? How can you do this?

Answer 38

melanoma –EMPD: pankeratin+, PAS+, S100- –Melanoma: pankeratin-, PAS-, S100+•

Question 39

Extramammary Paget’s Disease Gross: Micro: Tx

Answer 39

Gross: Crusting, weeping, oozing lesion; may be erythematous * Micro: large pale staining tumor cells, usually in lower epidermis, in nests, glandular spaces or continuously along basement membrane; contain mucin * TX: Complete surgical excision

Question 40

large pale staining tumor cells, usually in lower epidermis, in nests, glandular spaces or continuously along basement membrane; contain mucin: What tumur could this be? What markers could verify this assumption?

Answer 40

Extramammary Paget’s Disease ## Footnote +pankeratin +PAS+ -S100

Question 41

Crusting, weeping, oozing lesion

Answer 41

Extramammary Paget’s Disease

Question 42

* Papillary Hidradenoma * AKA Hidradenoma Papilliferum Gross: Micro

Answer 42

* May arise from apocrine sweat glands of vulva or from ectopic breast tissue along milk line * Gross: Benign, small, sharply circumscribed nodule covered by normal skin, often on labia majora or interlabial folds * Micro: well differentiated, complex papillary glandular pattern with some stratification and pleomorphism * TX: Excision

Question 43

arise from apocrine sweat glands of vulva or from ectopic breast tissue along milk line

Answer 43

Papillary Hidradenoma •AKA Hidradenoma Papilliferum

Question 44

•Condyloma acuminatum 1. Location: Related to what viral subtypes? A. B. C.

Answer 44

Sexual transmitted disease, lesions may be multiple and coalesce 1. Location: Vulvar, vaginal, perianal, perineal 2. HPV types 6 and 11

Question 45

•Condyloma acuminatum ## Footnote Gross Micro:

Answer 45

•Micro: acanthosis, hyperkeratosis, parakeratosis, nuclear atypia with raisin-like nuclei with perinuclear halos (“koilocytic change”). •Multiple treatment options

Question 46

Clear cell adenocarcinoma Symptoms? Gross: Micro: Tx

Answer 46

abnormal vaginal bleeding or discharge About 25% are asymptomatic Gross: usually a polypoid mass that typically originates from the anterior wall of the upper two-thirds of the vagina (may involve most of vagina); usually only superficially invasive at diagnosis Micro: malignant cells that have abundant clear cytoplasm (glycogen) and are arranged in a mixture of glandular, cystic, solid and papillary patterns, glands often contain hobnail cells. TX: Surgery, may need radiation therapy/chemotherapy for large/extensive tumors

Question 47

usually a polypoid mass that typically originates from the anterior wall of the upper two-thirds of the vagina (may involve most of vagina); usually only superficially invasive at diagnosis

Question 48

malignant cells that have abundant clear cytoplasm (glycogen) and are arranged in a mixture of glandular, cystic, solid and papillary patterns, glands often contain hobnail cells.

Answer 48

Clear cell adenocarcinoma