Vulvar Pathology Flashcards
1
Q
Non neoplastic disorders
A
- Ectopic mammary tissue
Inflammatory diseases
- Syphilis
- Granuloma inquinale
- Lymphogranuloma venereum
- Crohn’s disease
- Behcet’s disease
- Necrotizing fasciitis
- Vulvar vestibulitis
2
Q
Syphilis
A
- Chancre composed of plasma cells, lymphocytes and histiocytes
- Covered by a zone of ulceration infiltrated by neutrophils and necrotic debris
- Endarteritis: inflammation of arteries
3
Q
Granuloma inquinale
A
- Chronic infection caused by Calymmatobacterium granulomatis
- Begins as soft elevated granulomatous area which enlarges slowly by peripheral extension and ulcerates
4
Q
Granuloma inquinale histology
A
- Dense stromal infiltrate composed of histiocytes and plasma cells
- Scatterd small abscesses
- Donovan’s bodies: small round encapsulated bodies within the cytoplasm of histiocytes
- May spread retroperitoneum and stimulate a soft tissue neoplasm
5
Q
Lymphogranuloma venereum
A
- Caused by Chlamydia organism
- Affects lymph vessels and lymphoid tissue
- May have a small ulcer at the stie early
- Swelling of inguinal lymph nodes w/ stellate abscesses surrounded by pale epitheloid cells
- Scarring w/ fistulas and strictures of the vagina, urethra and rectum
6
Q
Crohn’s disease
A
- Assoc. w/ perineal disease and fistula formation
- Erythematous areas w/ ulceration
- May have noncaseating granulomas
7
Q
Behcet’s disease
A
- Autoimmune disease
- Vasculitis often involving the mucous membranes
- May have ocular problems
- Rare in vulva
- May present as nonspecific ulceration
- No cure, treatment focused on controlling symptoms
8
Q
Necrotizing fasciitis
A
- May be seen in diabetic women
- Assoc. w/ a high mortality rate
- Wide excision is the treatment of choice
9
Q
Vulvar vestibulitis
A
- Chronic inflammatory infiltrate
- Involves the lamina propria and periglandular connective tissue of the vestibular region
- Small glands in vestibule
- Can be inflamed
- Can produce severe pinpoint pain in vestibule
10
Q
Bartholin cyst
A
- Acute infection of Bartholin gland
- Produces acute inflammation
- Often assoc. w/ gonorrhea
- May cause abscesses
- Relatively common
- May become large 3-5cm
- Lined by transitional epithelium or squamous metaplasia
- Produce pain and discomfort
- Can be excised
- Cancer can occur and are mostly squamous cell ca
11
Q
Non neoplastic epithelial disorders
A
- Lichen sclerosis
- Squamous cell hyperplasia AKA Lichen simplex chronicus
12
Q
Lichen Sclerosis
A
- Atrophy of the epidermis w/ elimination of rete pegs
- Hydropic change of basal cells
- Replacement of dermis by dense collagen and just above that bandlike lymphocytic infiltrate
- Grossly presents as a white parchment-like patches
- Can occur at all ages
- Most common in postmenopausal pts
- May mimic sexual assault
- Assoc. w/ greater then expected risk of squamous cell carcinoma when assoc. w/ genetic alterations
13
Q
Squamous cell hyperplasia
A
- Caused by rubbing from pruritus
- Acanthosis and hyperkeratosis of vulvar epithelium
- May show increased mitotic activity
- Variable leukocytic infiltration of dermis
- Sometimes assoc. w/ ca
- May be caused by infections, chemical exposure, something that causes itching
- Biopsy to look for cellular differentiation and nuclear atypia
14
Q
Glandular neoplastic lesions
A
- Hidradenoma papilliferum
- Extramammary Paget Disease
15
Q
Hidradenoma Papilliferum
A
- Benign tumor presents as a well circumscribed nodule covered w/ normal skin
- Identical to intraductal papillomas of breast
- May arise from ectopoic breast tissue
- May ulcerate and mimic carcinoma
- Microscopically has a complex papillary structure w/ a myoepithelial layer
- May have some degree of pleomorphism
16
Q
Extramammary Paget Disease
A
- Pruritic, crusted sharply demarcated area
- Usually on labia majora
- May have palpable submucosal thickening
- Paget cells: arise from primitive epithelium, have a clear halo
- May persist for years w/o invasion
- Can mimic a lot of things, melanoma being one of them
17
Q
Benign Exophytic Lesions
A
- Condyloma Acuminatum
18
Q
Condyloma Acuminata
A
- Sexually transmitted
- Caused by HPV 6 and 11
- Benign
- Usually multiple and may coalesce
- Branchlike projections of squamous epithelium w/ a fibrous stroma
- Like many other wort-like processes it is assoc. w/ koilocytosis (perinuclear halo)
- Frequently regresses spontaneously
- Not a precancerous lesion
- Marker for STD
19
Q
Squamous neoplastic lesions
A
- Vulvar intraepithelial neoplasia
- Vulvar carcinoma: invasive, microinvasive and verrucous carcinoma
20
Q
Vulvar intraepithelial neoplasm (classic type)
A
- Assoc. w/ high risk HPV
- Characterized by nuclear atypia, increased mitoses
- Grossly presents as white or pigmented plaques
- Often multicentric
- 90% of VIN contains HPV 16 and 18
- Lack of surface differentiatons
21
Q
Vulvar intraepithelial neoplasm (differentiated variant)
A
- Assoc. w/ squamous cell hyperplasia and lichen sclerosis
- Not typically assoc. w/ HPV
- P53 overexpression
- Cancer may develop quickly as nodule in a background of inflammation
- Invasive form has keratinization
22
Q
Vulvar Cancer
A
- Squamous cell cancer makes up 95% of vulvar cancer
- Differentiated and Classic type
- Age at presentation 60-74
- Mostly in labia majora
- Tumors of labia metastasize to lymph nodes
- Tumors of clitoris metastasize directly to deep lymph nodes
23
Q
Vulvar Cancer Risk Factors
A
- Number of lifetime sex partners, cigarette smoking, and immunodeficiency
24
Q
Vulvar Cancer Differentiated type
A
- Older women, not assoc. w/ HPV, keratinizing
25
Vulvar Cancer Classic type
- Younger women, **assoc. w/ HPV** often w/ a warty histology
26
Vulvar Cancer Prognosis and Prognostic Factors
- Prognosis: 5 year survival 50-75%
- Prognostic factors: tumor diameter, LN status and depth of invasion
27
Microinvasive Vulvar Cancer
- Vulvar Ca w/ depth of penetration less than 5mm
- Often have low incidence of LN metastasis
- Presence of eosinophils in VIN may be a clue to early invasion
28
Verrucous Carcinoma
- Type of squamous carcinoma
- May be large, exophytic and infiltrates locally
- Mets are almost non existent
- Can mimic condyloma acuminata and conventional squamous cell carcinoma
- Does not have cytologic atypia and or a clearly infiltrative pattern of growth like that seen in conventional squamous cell carcinoma
29
Aggressive angiomyxoma
- Mimics a Bartholin gland cyst
- Most women are in 2nd or 3rd decade of life
- Grossly edematous ill defined mass
\*soft, gelatinous, encapsulated
- Hypocellular stroma w/ little atypia or mitotic activity w/ large blood vessels
- Recurrence is common
30
Aggressive angiomyxoma Histology
31
Melanoma
- Occurs especially in labia majora
- 2nd most common malignant tumor of vulva
- Most pts are \>50
- Most lesions are advanced by time of diagnosis
- LN status, depth of penetration, and ulceration are prognostic factors
- 5 year survival ~35%
32
Page disease vs Melanoma
- **Paget**: pos. for PAS, keratin, mucicarmine, alcian blue
- **Melanoma**: pos. for S100 and HMB 45
33
Vaginitis
- Vaginal discharge
- Candida albicans: curdy white discharge
- Trichomonas vaginalis: copious gray green discharge
34
Benign epithelial tumors
- **Intramural papilloma**: branching configuration w/ a lining of a single layer of cuboidal cells
- **Squamous papilloma**: most are due to HPV
- **Tubulovillous adenoma**: similar to colorectal type
- **Benign mixed tumor**: made of stromal-type spindle glands mixed w/ mature squamous cells and glands lined by mucinous epithelium
35
Vagina intraepithelial neoplasms and vaginal ca
- Uncommon, usually squamous cell CA
- Usually in women \>60
- Most arise from extension of cervical squamous cell CA but can arise as a primary tumor
36
Clear cell adenocarcinoma
- Usually occurs in the anterior or lateral wall of the upper vagina
- Avg age of diagnosis 17
- In 2/3 of pts. there is a history of prenatal exposure to **diethylstilbesterol (DES)** or related nonsteroid estrogens
- **Vaginal bleeding** or discharge are common symptoms
37
Mesenchymal tumors and tumorlike lesions
- **Fibroepithelial polyps**: may be seen in adult women or neonates, have a fibrovascular core line by squamous epithelium
- **Leiomyoma**: most common benign mesenchymal tumor of the vagina
- **Leiomyosarcoma**: can be large and ulcerate, moderate to marked atypical cells
- **Rhabdomyoma**: polypoid mass, seen in adults, haphazardly arranged spindle cells w/ a few mitoses
38
Sarcoma Botryoides
- AKA botryoid rhabdomyosarcoma
- Rare
- Polypoid invasive tumor arises from the anterior vaginal wall
- Most cases are in girls \<5 w/ many during first 2 years
- Soft polypoid masses resembling a bunch of grapes
- Myxoid stroma w/ round or spindle cells
- Crowding of cells around blood vessels
