Flashcards in Pathology: Vulva, Vagina, & Cervix Deck (18)
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1
What is the vulva? What type of cell lines it?
- the vulva is the skin of the genitalia external to the hymen
- it is lined by squamous epithelium
2
What is a Bartholin cyst? How do patients present?
- this is a cystic dilation of a Bartholin gland (lubricates the vestibule) due to inflammation and/or obstruction
- patients present with a unilateral painful lesion in the lower vestibule, just adjacent to the vagina
3
What is a condyloma? What is it due to? Does it increase the risk for carcinoma?
- a condyloma is a warty neoplasm due to infection with HPV serotypes 6 and 11 (these are the low-risk serotypes; types 16, 18, 31, and 33 are the high-risk ones)
- these can occur anywhere in the lower genital tract (vulva, vagina, cervix)
- it can very rarely progress to carcinoma
4
What type of cellular change is associated with an HPV infection?
- koilocytic histology
- cells contain a clear halo around a crinkled pyknotic nucleus that resembles a raisin, and increased mitotic activity
5
What is lichen sclerosis? What about lichen simplex chronicus? How do patients present? Do either increase the risk for carcinoma?
- lichen sclerosis: thinning of the epidermis and fibrosis of the underlying dermis; patients present with leukoplakia and parchment-like (very thin) vulvar skin
- lichen simplex chronicus: hyperplasia of the squamous epithelium; patients present with leukoplakia and thick, leathery vulvar skin
- ONLY lichen sclerosis is associated with a (slightly) increased risk of developing squamous cell carcinoma of the vulva
6
How do patients with vulvar carcinoma present? What are the major differentials for this presentation? What are the two pathways for vulvar carcinoma to develop?
- patients with vulvar carcinoma present with leukoplakia
- this is the same presentation as lichen sclerosis (slightly increased risk of malignancy) and as lichen simplex chronicus (completely benign); therefore, a biopsy is needed
- 2 pathways: via HPV (serotypes 16, 18, 31, 33) or via long-standing lichen sclerosis
- (patients with HPV carcinoma are usually younger, 40-50; patients with non-HPV carcinoma are usually older, 70+)
- note that vulvar carcinoma is relatively rare (occurrence: endometrial then ovarian then cervical then vulvar and vaginal)
7
What is extramammary Paget disease? How does it present? What is the major differential and how do we rule it out?
- this is the presence of malignant epithelial cells in the epidermis of the vulva (it is called extramammary because it usually occurs in the nipple)
- it is associated with carcinoma in situ (nearly 100% association when in the nipple, but most cases in the vulva don't involve cancer)
- presents as erythematous, pruritic, ulcerated skin
- must be distinguished from melanoma:
- Paget: PAS positive, keratin positive, S100 negative
- melanoma: PAS negative, keratin negative, S100 positive
8
What is adenosis and what is is highly associated with? What is rare but serious complication of this disease?
- adenosis is the focal persistence of columnar epithelium in the upper 1/3 of the vagina (in the fetus: the lower 2/3 of the vagina is squamous epithelium and derived from the uro-genital sinus, the upper 1/3 is columnar and derived from the Mullerian duct; normally, the upper 1/3 gets replaced by squamous epithelium during development)
- highly associated with DES (a drug no longer in use) exposure in utero
- rare complication: vaginal clear cell adenocarcinoma
9
What is embryonal rhabdomyosarcoma? What cell type is involved? What is it also known as? How do patients present?
- this is a very rare malignant mesenchymal proliferation of immature skeletal muscle cells (the rhabdomyoblast) of the vagina
- it is AKA sarcoma botryoides because of it's grape-like physical appearance
- patients present as children with vaginal bleeding and a grape-like mass protruding from the vagina
10
What features/markers characterize rhabdomyoblasts?
- (rhabdomyoblasts are the immature skeletal muscle cells involved in the malignant proliferation known as embryonal rhabdomyosarcoma)
- these cells have cytoplasmic cross-striations and have positive IHC staining for desmin (intermediate filaments found in skeletal muscle) and myoglobin
11
What causes vaginal carcinoma? Which lymph nodes does the cancer tend to spread to?
- vaginal carcinoma is relatively rare (occurrence: endometrial then ovarian then cervical then vulvar and vaginal)
- is it usually related to infection with high-risk HPV (16, 18, 31, 33), in which case it develops from the precursor lesion VAIN (vaginal intraepithelial neoplasia)
- carcinoma of the lower 2/3 of the vaginal canal (derived from the urogenital sinus) spreads to the inguinal lymph nodes
- carcinoma of the upper 1/3 (derived from the Mullerian duct) spreads to the regional iliac lymph nodes
12
Where is the most common site of HPV infection?
- (HPV can infect the lower genital tract: vulva, vagina, and cervix)
- it most commonly infects the cervix at the site of the transformation zone (when the exocervix becomes the endocervix; stratified squamous becomes columnar)
13
What action of HPV causes the intraepithelial neoplasia? Which serotypes are involved?
- this involves the high-risk HPV serotypes: 16, 18, 31, and 33, as these cause the dysplasia that can progress into carcinoma
- these serotypes have an E6 and E7 virulence factor; E6 destroys p53 and E7 destroys Rb
- this results in intraepithelial neoplasia (in the cervix this is CIN, vagina: VAIN, vulva: VIN)
14
How do we classify the level of intraepithelial neoplasia in the cervix due to HPV infection? Which classifications are still reversible?
- classification of CIN is based on how much of the cervical wall is involved in the dysplasia
- CIN 1: 1/3 is dysplastic; 66% chance of reversal
- CIN 2: 2/3 is dysplastic; 33% chance of reversal
- CIN 3: greater than 2/3; unlikely to reverse but possible
- carcinoma in situ (CIS): whole wall; neoplastic, so no chance of reversal, will likely invade the basement membrane to become cervical carcinoma
15
What is the major risk factor for developing cervical carcinoma? What are two other risk factors? What is this cancer's relationship to HIV/AIDS?
- major risk factor is persistent infection with high-risk HPV serotypes 16, 18, 31, or 33
- other risk factors: smoking and immunodeficiency
- cervical carcinoma is one of the AIDS defining illnesses
16
How do patients with cervical carcinoma classically present? How do advanced tumors present?
- most common age group is 40-50 (patients get infected in their 20's and the dysplasia and neoplasia develop slowly over the next 20 or so years)
- presents as abnormal vaginal bleeding, malodorous discharge, and postcoital bleeding
- cervical carcinoma tends to grow locally and invade local structures rather than metastasize, so advanced tumors often invade the bladder; patients present with blockage of ureters and hydronephrosis with eventual post-renal azotemia/failure (renal failure is actually a major cause of death in these patients)
17
What are the two types of cervical carcinoma? How do we detect this carcinoma?
- 2 types: squamous cell (much more common; 85%) and adenocarcinoma (15%)
- BOTH types are associated with HPV
- we screen for cervical carcinoma with the pap smear (used to determine the CIN classification); the pap smear is VERY good at detecting squamous cell carcinoma, but is not nearly as good for adenocarcinoma
- positive pap smears are followed up with colposcopy and biopsy
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