STEPTRACKERREPRODUCTIVE | PATHOLOGY + MICROBIOLOGY | HIGH-YIELD REVIEW

Vulva & Vagina

White patches, warts, two roads to vulvar cancer, Paget cells, a DES-linked carcinoma and the grape-like tumor of little girls.

FOCUS ON
Lichen sclerosus: thin white parchment skin, older women, small SCC risk. Vulvar SCC: HPV-related (younger, from VIN) vs HPV-independent (older, lichen sclerosus, TP53). Extramammary Paget: intraepithelial adenocarcinoma, usually no underlying cancer. DES → clear cell adenocarcinoma. Sarcoma botryoides: child < 5, grape-like vaginal mass.
LESION
MECHANISM · CAUSE
FEATURES
CLUE
Bartholin cyst / abscess
Obstructed Bartholin duct; infection (polymicrobial, gonococcus, chlamydia)
Painful unilateral swelling at the lower lateral vestibule; reproductive age
Drainage (Word catheter, marsupialization)
Lichen sclerosus
Probably autoimmune; epidermal thinning, dermal fibrosis
Thin white "parchment-like" plaques, itching; postmenopausal women; also prepubertal girls
Slight ↑ SCC risk · potent topical steroids
Lichen simplex chronicus
Chronic rubbing/scratching → squamous hyperplasia
Thick, leathery white plaques
No ↑ cancer risk
Condyloma acuminatum
Low-risk HPV 6, 11
Soft, warty, cauliflower lesions; koilocytes (raisinoid nuclei, perinuclear halo)
Rarely progresses · vs condyloma lata (syphilis)
HPV-related vulvar SCC
High-risk HPV 16/18 → usual-type VIN → SCC
Women 40–50s; often multifocal; smokers, immunosuppression
p16 ⁺
HPV-independent vulvar SCC
Lichen sclerosus → differentiated VIN; TP53
Older women (70s); single lesion
Spreads to superficial inguinal nodes
Extramammary Paget disease
Intraepithelial adenocarcinoma cells in the epidermis (likely from apocrine/stem cells)
Red, crusted, itchy, eczema-like vulvar plaque; large pale cells with mucin
Keratin/CK7 ⁺, PAS ⁺; S100 ⁻ · usually no underlying carcinoma
Vulvar melanoma
Malignant melanocytes
Pigmented or nonpigmented lesion; older women
S100, HMB-45 ⁺; keratin ⁻ (vs Paget)
Vaginal clear cell adenocarcinoma
Diethylstilbestrol (DES) in utero → vaginal adenosis (glandular columnar tissue) → carcinoma
Young women; also T-shaped uterus, cervical anomalies
Clear cells with glycogen
Vaginal SCC
Usually extension of cervical SCC; primary is rare (HPV)
Upper vagina → iliac nodes · lower vagina → inguinal nodes
Think cervix first
Embryonal rhabdomyosarcoma (sarcoma botryoides)
Malignant skeletal-muscle precursor tumor
Girls < 5: bleeding, grape-like mass protruding from the vagina; cambium layer under epithelium
Desmin, myogenin ⁺

Mechanism — two roads to vulvar SCC

High-risk HPVE6 → p53 · E7 → Rb
→
Usual VINp16 ⁺
→
SCCYounger, multifocal
Lichen sclerosusChronic inflammation
→
Differentiated VINTP53 mutation
→
SCCOlder, solitary

Vulva and lower vagina drain to superficial inguinal nodes; upper vagina to internal/external iliac nodes.

Vaginitis compared

CAUSE
DISCHARGE
pH
CLUE · DRUG
Bacterial vaginosis
Thin gray, fishy
> 4.5
Clue cells, ⁺ whiff · metronidazole
Trichomonas
Frothy yellow-green
> 4.5
Motile flagellates, strawberry cervix · metronidazole (treat partner)
Candida
Thick white, "cottage cheese"
Normal (≤ 4.5)
Pseudohyphae on KOH · azoles

How it's tested

COMMON CONFUSIONExtramammary Paget and melanoma both show large pale cells in the epidermis. Paget is keratin ⁺ and mucin ⁺; melanoma is S100 ⁺ and HMB-45 ⁺.
THE TRAPPaget disease of the nipple nearly always means an underlying breast carcinoma; extramammary (vulvar) Paget usually does not.
YOUR APPROACHAge (child, young, older) → color and texture (white, warty, red-crusted, grape-like) → exposure (HPV, DES) → stain → node drainage.

If you see… think…

Postmenopausal woman with thin white parchment-like vulvar skin → lichen sclerosus
Painful lump at the lower lateral vaginal opening → Bartholin abscess
Cauliflower vulvar warts with koilocytes → condyloma acuminatum (HPV 6, 11)
Woman in her 40s, smoker, multifocal p16⁺ vulvar SCC → HPV-related SCC
Woman in her 70s with lichen sclerosus and a vulvar ulcer → HPV-independent SCC
Red, itchy, crusted vulvar plaque with keratin⁺ pale cells → extramammary Paget disease
Young woman with vaginal mass, mother took DES → clear cell adenocarcinoma
3-year-old girl with grape-like mass from the vagina → sarcoma botryoides
Fishy discharge, clue cells, pH 5.5 → bacterial vaginosis
Frothy green discharge, strawberry cervix → Trichomonas