L44- Female GUT Pathology III (vagina)

Question 1

Vagina:

• (1) size
• (2) simple definition, anatomically
• (3) embryonic origin

Answer 1

1- 2.5-3 in long

2- muscular tube between vulva and cervix

3:

• Mullerian ducts – upper 3/4ths vagina
• Urogenital sinus – lower 1/4th vagina

Question 2

Uterine didelphys: definition and origin

Answer 2

• two uteri, two cervices, two vaginas

- TOTAL (not partial) failure of mullerian ducts to fuse

Question 3

list the vaginal lesions (not vulva)

Answer 3

• Gartner’s duct cyst
• Adenosis, clear cell carcinoma
• SCC

Question 4

list the vulva lesions (not vagina)

Answer 4

• Bartholin’s cyst
• nonneoplastic epithelial disorders
• condyloma acuminatum (genital warts)
• extramammary Paget’s disease
• VIN, SCC

Question 5

Gartner’s duct cyst:

• (1) size and location
• (2) origin / cause

Answer 5

1- anterolateral wall of vagina, 1-2 cm

2- mesonephric / wolffian ducts

Question 6

Vaginal Adenosis is seen in girls at (1) age, with mothers with a (2) history. (3) is the development seen in the vaginal wall due to (4) dysfunction. (5) is the risk in some females.

Answer 6

1- 10 y/o
2- diethyl stillbesterol (DES) regimen during pregnancy to prevent abortion
3- endocervical type glands
4- inhibition of Mullerian epithelial transformation into squamous epithelium
5- clear cell adenocarcinoma (10-35 y/o)

Question 7

SCC of the Vagina:

• (un-/common)
• (2) cause, with (3) lesion initially
• (4) mass description
• (5) is also evaluated
• (good/poor) prognosis

Answer 7

1- uncommon
2- high risk HPV [16, 18, 31, 33]
3- vaginal intraepithelial neoplasia (VIN 1,2,3)
4- exophytic, polyploidal, fungating mass
5- pelvic / inguinal LNs
6- poor prognosis

Question 8

Sarcoma Botryoides:

• aka (1)
• (2) affected age group
• (3) general appearance (based on name)

Answer 8

1- embryonal rhabdomyosarcoma
2- <5y/o
3- Botryoides = ‘bunches of grapes’ hanging w/in vagina

Question 9

Sarcoma Botryoides:

• aka (1)
• (low/high) malignant
• (3) microscopic appearance
• (4) type invasion / spread
• (5) Tx

Answer 9

1- embryonal rhabdomyosarcoma
2- highly malignant
3- maligant rhabdomyoblasts, cambium layer, fibromyxomatous
4- local invasion
5- surgery, chemotherapy

Question 10

list the components of the vulva

Answer 10

• labia majora, minora
• vestibule
• clitoris
• Bartholin glands (open into vaginal introitus/opening)
• Skene glands (around urethral opening)

Question 11

Vulvar Vestibule components

Answer 11

• vaginal opening = introitus
• urethral opening
• major vestibular / Bartholin’s glands
• minor vestibular glands, periurethral Skene’s ducts (homologues of prosate gland, immediately adjacent and posterolateral to urethral)

Question 12

Bartholinitis:

• (1) definition resulting from (2)
• (3) causes
• (4) and (5) may develop
• (6) Tx

Answer 12

1- acute inflammation, inferior labia majora
2- Batholin gland obstruction

3- Strep. spp, Staph. spp, Gonococci, E. Coli

4- vaginal blocking
5- abscess formation
6- drainage and marsupialization (special surgery)

Question 13

Condyloma acuminatum = _____ + description

Answer 13

Genital Warts: bulky, warty growth

• possibly multiple
• hyperplasia, koilocytosis

Question 14

Leukoplakia:

• (1) definition
• (2) causes
• (3) clinical importance

Answer 14

1- white plaques associated with pruritus and scaling

2: (neoplastic and non-neoplastic)
- inflammatory dermatoses (psoriasis)
- Lichen sclerosus, squamous cell hyperplasia
- Neoplasia: vulva intraepithelial neoplasia (VIN), Paget’s disease, invasive carcinoma

3- biopsy to r/o neoplastic cause

Question 15

Lichen sclerosus = (1):

• (2) common age of onset
• (3) type disease pathogenesis
• (4) risk of carcinoma

Answer 15

1- Kraurosis vulvae

2- postmenopausal

3- autoimmune nature

4- slightly inc risk of carcinoma over leukoplakia alone

Question 16

Lichen sclerosus morphological changes

Answer 16

• thinning of epidermis
• Rete Peg disappearance
• Dermal fibrosis (dense collagen)
• band-like inflammatory infiltrates

-**Leukoplakia: scaly white plaques, parchment like

Question 17

Squamous Cell Hyperplasia on vulva = (1):

• (2) common age of onset
• associated with (3) symptom
• (4) malignant potential

Answer 17

1- Lichen simplex chronicus
2- post-menopausal
3- pruritus and chronic irritation of vulva
4- NONE

Question 18

Squamous Cell Hyperplasia of vulva morphological changes

Answer 18

• acanthosis, localized hyperplastic epidermis, hyperkeratosis
• inflammatory infiltrates
• inc mitotic activity, no atypia

Question 19

Paget disease of vulva:

• (1) = primary
• (2) = secondary

Answer 19

1- adenocarcinoma in-situ or invasive

2- due to underlying malignancy –> GYN, GI, GU in 15-30% cases

Question 20

list VIN risk factors (include age), classic type

Answer 20

(vulvar intraepithelial neoplasia)

• HPV 16, 18, 31, 33 (high risk)
• mean age ~40 y/o
• smoking
• immunosuppressed patients

Question 21

VIN, classic type:

-(1) is main appearance with a (2) distribution

Answer 21

(vulvar intraepithelial neoplasia)
1- Leukoplakia, reddish brown plaque

2:

• 50-80% multifocal
• 50-60% synchronous lesions in cervix, vagina, urethra, anus

Question 22

VIN, classic type:

• (1) odds of recurrence after local Tx, more likely in (2) patients
• (3) risk of invasion after Tx
• (4) is the ultimate Tx

Answer 22

(vulvar intraepithelial neoplasia)
1- 35-50%
2- younger patients
3- 4-7%
4- surgical excision

Question 23

VIN, differentiated type:

• (1) main cause
• (2) mainly affected group

Answer 23

(vulvar intraepithelial neoplasia)
1- p53 mutation (not HPV)
2- older women

Question 24

VIN, differentiated type appearance / make-up

Answer 24

(vulvar intraepithelial neoplasia)

• basal atypical layer - basal cell atypia
• maturation of superficial layers, hyperkeratosis
• in-site lesion w/o invasion

Question 25

Carcinoma of Vulva: - (1) most affected group - mostly (2) type in (3) location and (4) character - (5) are also investigated in these cases

Answer 25

``` 1- >60y/o 2- SCC: keratinizing, basiloid types (keratin pearls, intracellular bridges) 3- anterior 2/3 labia majora 4- plaque or nodule or ulcer 5- inguinal and pelvic nodes ```

Question 26

Vulva SCC, keratinizing type: - (more/less) common than than basaloid SCC - mostly (older/younger) females - (uni/multi)-focal - (rare/common) associated multifocal lower GUT neoplasia - (5) morphology - (rare/common) associated VIN - (7) associated HPV - (rare/common) associated vulvar dystrophy - (9) IHC markers

Answer 26

``` 1- more, 80% 2- older 3- usually unifocal (or multifocal) 4- rare 5- keratinizing 6- rare (differentiated type) 7- HPV-β (cutaneous)- 5, 8 8- common 9- p53+ (some cases), p16(+/-) ```

Question 27

Vulva SCC, basaloid type: - (more/less) common than than keratinizing SCC - mostly (older/younger) females - (uni/multi)-focal - (rare/common) associated multifocal lower GUT neoplasia - (5) morphology - (rare/common) associated VIN - (7) associated HPV (+ type) - (rare/common) associated vulvar dystrophy - (9) IHC markers

Answer 27

``` 1- less, 25% 2- younger 3- multifocal 4- common 5- worly 6- common (classic type) 7- HPVα- 16 > 18 8- rare 9- p53-, p16+ ```

Question 28

list the different Gestational trophoblastic disease- **indicate the main marker

Answer 28

- Hydatidiform mole- complete/incomplete - invasive mole - gestational choriocarcinoma - placental site trophoblastic tumor -**β-hCG in urine, serum is critical marker

Question 29

define hydatidiform mole

Answer 29

Uterus: - filled with translucent grape-like clusters of edematous, distorted structures - deficiency of fetal BVs (no fetal development)

Question 30

Hydatidiform mole: - (1) age/geographic risk factors - (2) types - (3) 3 key characteristics

Answer 30

1- extremes of age, Far East countries 2- partial or complete 3: i) large edematous, avascular villi ii) stroma degeneration iii) trophoblast proliferation

Question 31

Complete Mole: - (1) genomic status of ovum - (2) morphological changes - (3) physiological marker - small risk of developing (4)

Answer 31

1- loss of chromosomes of ovum --> fertilized by 2 sperm or 1 diploid sperm => mostly 46XX, rarely 46XY 2- (embryo dies early, no fetal parts seen) enlarged uterus filled with grape like vesicles 3- β-hCG (may produce bilateral theca lutein cysts) 4- choriocarcinoma (2%)

Question 32

Partial Mole: - (1) genomic status of ovum - (2) morphological changes - (3) malignancy risk

Answer 32

1- fertilized by two (23X, 23Y) sperms => triploid (69XXY) mole 2- (short embryo development, some fetal parts seen) minimally enlarged uterus 3- no risk of malignancy

Question 33

Hydatidiform mole: - (1) main signs and Sxs - (2) can develop after 3-4 mos

Answer 33

1- amenorrhea (enlarged uterus), vomiting, positive pregnancy test 2- vaginal bleeding, grape-like structures

Question 34

Hydatidiform mole: - (1) US appearance - (2) physiological test - (3) Tx - (4) monitoring

Answer 34

1- snowstorm appearance, absent fetus if complete 2- elevated β-hCG, serum/urine 3- curettage for lesion removal 4- β-hCG levels (10% persistent/invasive, 2% choriocarcinoma)

Question 35

Invasive mole: - (1) frequency - (2) definition - (3) associated features - (4) is important risk / development - (5) Tx

Answer 35

1- 10% of complete moles 2- deep invasion of villi, trophoblasts --> myometrium 3- necrosis, hemorrhage of myometrium 4- uterine rupture (requires hysterectomy) --> villi embolize in the lung (regress) 5- chemotherapy

Question 36

Gestational Choriocarcinoma: - (1) geographic risk - 50% develop from (2), 25% from (3)

Answer 36

1- more in Asia, Africa than USA 2- hydatidiform mole 3- abortion, normal pregnancy, ectopic pregnancy

Question 37

Gestational Choriocarcinoma: - (1) mass description - (2) usual presentation - (3) Tx

Answer 37

1- not bulky, uterine bleeding 2- advanced presentation --> lung, vagina, brain, kidney affected 3- evacuation + chemotherapy

Question 38

Gestational Choriocarcinoma: - (1) are malignant, (2) are absent - (3) is initial screening followed by (4) - (5) monitoring

Answer 38

1- cytotrophoblasts, syncitiotrophoblasts 2- chorionic villi 3- β-hCG elevated 4- biopsy 5- β-hCG