Pathology of Vulva & Vagina Flashcards
What is a bartholin cyst?
- cystic dilation of the bartholin gland (produces mucus-like fluid that drains via ducts into the lower vestibule) that arises due to inflammation and obstruction of the gland in women of reproductive age.
How does a batholin cyst present?
- as a unilateral, painful cystic lesion at the lower vestibule adjacent to the vaginal canal.
** What is a condyloma?
- a watery neoplasm of vulvar skin that is often large.
- most commonly due to HPV 6 and 11 (CONDYLOMA ACUMINATA); LOW RISK.
- less commonly due to secondary syphilis (CONDYLOMA LATUM).
- both sexually transmitted
- HPV condyloma characterized by KOILOCYTES (hallmark of HPV cells; nucleus looks crinkled like a raisin with perinuclear HALO).
What does HPV affect?
- the lower female genital tract (vulva, lower vaginal canal, and cervix)
Do condylomas often progress to carcinoma?
NO :)
What are the high risk HPV subtypes?
- 16, 18, 31, 33.
** What is lichen sclerosis? (BOARDS LOVE THIS)
- THINNING of the epidermis and fibrosis (sclerosis) of the dermis (replaced by DENSE collagen).
- presents as a white patch (leukoplakia) with PARCHMENT-LIKE vulvar skin.
- BENIGN
- most commonly seen in POSTMENOPAUSAL women (or from sexual assault).
What causes lichen sclerosis?
possibly autoimmune
Is lichen sclerosis associated with a slightly increased risk for squamous cell carcinoma?
YES
What is lichen simplex chronicus (squamous cell hyperplasia)?
- hyperplasia of the vulvar squamous epithelium.
- presents as leukoplakia with THICK, leathery vulvar skin.
- associated with CHRONIC IRRITATION and SCRATCHING.
- BENIGN
Is there an increased risk for squamous cell carcinoma in lichen simplex chronicus?
NO :)
What is vulvar carcinoma?
- carcinoma arising from squamous epithelium lining the vulva.
- rare
- presents as leukoplakia; biopsy to distinguish from other leukoplakia causes.
** What is the etiology of vulvar carcinoma?
- can be HPV related (due to high-risk types 16 and 18)= arises from VIN (vulvar intraepithelial neoplasia), a dysplastic precursor lesion characterized by KOILOYTIC change. Occurs around ages 40-50.
- can be NON-HPV related= usually from long-standing lichen sclerosis around age 70.
What is extramammary Paget disease?
- malignant epithelial cells in the epidermis of the vulva (usually labia majora).
- presents as erythematous, pruritic, ulcerated vulvar skin.
- represents CARCINOMA IN SITU, usually with no underlying carcinoma.
- Paget disease of the nipple can also occur, but is almost ALWAYS with an underlying carcinoma of the breast.
*** From what must you distinguish extramammary Paget disease (i.e. carcinoma)?
- MELANOMA= PAS-, keratin-, and S100 +
- Paget cells= PAS+ (indicates that cells are making mucus and only epithelial cells make mucus), keratin+ (intermediate filament present in epithelial cells), and S100-. Also have a clear halo.
Where can ectopic mammary tissue occur?
- along primitive milk line. Tissue is subject to physiologic and pathologic changes including swelling and secretion of milk during pregnancy.
What does syphilis do?
- produces a chancre (HARD ulcer) with inflammatory infiltrate composed of plasma cells, lymphocytes, and histiocytes. This is covered by a zone of ulceration on the vulva infiltrated by neutrophils and necrotic debris.
- syphilis also has a predilection for small blood vessels causing ENDARTERITIS (especially vaso vasorum of aorta).
What is granuloma inguinale?
- chronic infection caused by Calymmatobacterium granulomatis.
- begins as a SOFT elevated granulomatous area, which enlarges slowly and has dense stromal infiltrate.
- characterized by DONOVAN’S bodies.
What is lymphogranuloma venereum?
- swelling of inguinal lymph nodes with stellate abscesses surrounded by pale epitheliod cells.
- caused by Chlamydia.
- may have a small ulcer at the site early.
What disease is associated with PERINEAL disease and fistula formation?
- Crohn’s disease (aka GI involvement)
What is Behcet’s disease?
- autoimmune vasculitis involving the mucous membranes.
- may have ocular problems, but rare in vulva.
What is vulvar vestibulitis?
- chronic inflammatory infiltrate involving the lamina propria and periglandular connective tissue of the vestibule.
- small glands can be inflamed, producing severe pinpoint pain in vestibule.
What is Hidradenoma papilliferum?
- BENIGN tumor that presents as a well circumscribed nodule covered with normal skin.
- identical to intraductal papillomas of breast.
- may arise from ectopic breast tissue.
What do we have to know about the differentiated variant of VIN?
- associated with squamous cell hyperplasia (lichen simplex chronicus) and lichen sclerosis.
- p53 overexpression
- NOT typically associated with HPV
What is microinvasive cancer?
- vulvar cancer with depth of penetration less than 5 mm.
- often have low incidence of lymph node metastasis.
What is verrucous carcinoma?
- type of squamous carcinoma that may be large, exophytic (grows out of vulva) and infiltrates locally.
- mets nonexistent :)
- can mimic condyloma
What is aggressive angiomyxoma?
- mimics bartholin gland cyst usually in women in their 20s-30s.
- grossly edematous with hypocellular stroma (not many cells there, but large blood vessels).
- recurrence is common
What type of epithelium lines the vagina?
- NON-keratinized stratified squamous epithelium
What is Adenosis?
- focal persistence of columnar epithelium in the upper vagina (due to lack of stratified squamous epithelium replacement during development).
What females are at risk for adenosis?
- those who were exposed to DES (diethylstilbestrol) in utero; estrogen compound used for pregnancy related complications, but crosses the placenta.
** What is the problem with adenosis? (BOARDS LOVE THIS)
- can lead to CLEAR CELL ADENOCARCINOMA= malignant proliferation of glands with clear cytoplasm. Rare. Can also cause infertility in daughters of DES moms.
For what does DES increase a mother’s risk?
breast cancer
** What is embryonal rhabdomyosarcoma (sarcoma botryoides)?
- malignant mesenchymal proliferation of immature skeletal muscle; rare.
- presents as bleeding and a GRAPE-LIKE mass protruding from the vagina or penis of a child (less than 5 y/o).
What key cell forms the rhabdomyosarcoma?
rhabdomyoblast= exhibits cytoplasmic cross-striations and + immunohistochemical staining for DESMIN (intermediate filament present in muscle cells) and MYOGENIN (nuclear transcription factor present in immature muscle).
What is vaginal carcinoma?
- carcinoma arising from squamous epithelium lining the vaginal mucosa.
- usually related to high-risk HPV (16 & 18)
- precursor lesion is VAGINAL intraepithelial neoplasia (VAIN; not VIN).
** What is important to remember about vaginal carcinoma spread to the lymph nodes?
bc the vagina is derived from 2 different embryological sources the lower 1/3 (derived from the urogenital sinus) will spread to INGUINAL nodes, and the upper 2/3 (derived from the mullerian duct) will spread to regional ILIAC nodes.
What will benign mixed tumors of the vagina have?
- made of stromal-type spindle glands mixed with mature squamous cells
What are fibroepithelial polyps of the vagina?
- mesenchymal tumor that has a fibrovascular core lined by squamous eptihelium.
What is the most common BENIGN MESENCHYMAL tumor of the vagina?
leiomyoma
