Vulva
Vulva
Vulva
Hidradenoma Papilliferum
Benign. Often presents as an
asymptomatic nodule.
Virtually identical to intraductal
papilloma of the breast
Well-circumscribed subepithelial nodule
Papillary proliferation with tubular glands
Apocrine differentiation with apical snouts
Two cell layers (inner epithelial and outer
myoepithelial) can be seen on IHC.
Condyloma acuminatum→ grossly evident variant of LSIL. Often composed of papillary fronds.
Squamous Intraepithelial Lesion (SIL) (Continued…)
High-grade Squamous Intraepithelial Lesion (HSIL)
Associated with High-risk HPV (usually type 16). Higher risk
of progression to invasive carcinoma if left untreated
compared to LSIL, but not super high absolute risk.
Proliferation of hyperchromatic basal-like cells that extend
2/3 of the way up (VIN2) or full-thickness (VIN3/CIS) of the
epithelium
Cells have enlarged, hyperchromatic nuclei with irregular
nuclear contours and increased N:C ratios.
Little to no superficial maturation.
Mitoses common at all levels, including atypical mitoses
Nucleoli are unusual→ raise the possibility of inadequately
sampled invasive carcinoma (p16+) or metaplasia (p16-)
CK7 CK20 GCDFP-15 CDX2 CEA S100, MelanA, UPK III HER2 GATA-3
etc…
Primary
Paget Disease + - + - + - - + +
Urothelial
carcinoma + + - - - - + - +
Anorectal
carcinoma +/- + - + + - - - -
Melanoma - - - - - + - - -
Squamous Cell Carcinoma
An invasive epithelial tumor composed of squamous cells with varying degrees of differentiation.
Derived from HSIL (HPV-related) or Differentiated VIN (not HPV-related)
Most common vulvar malignancy. Most common in elderly.
Most important factor determining outcome→ Lymph node status
Most important factor determining Lymph node metastases→ depth of invasion
Femoral and inguinal lymph nodes are the sites of regional spread
Sheet-like growth with infiltrating bands and single cells
Often desmoplastic/inflammatory stroma
Two main morphologic types:
Keratinizing Basaloid
Squamous Squamous
Carcinoma Carcinoma
High-risk HPV No Yes (Type 16>18)
association
Other Tumors
Melanocytic nevi—Like nevi elsewhere on the skin, but remember the vulva is a “special site.” As
such, there can be concerning (but benign) changes including Pagetoid spread, moderate cytologic
atypia, an adnexal spread. There should be dermal maturation and no dermal mitoses.
Melanoma—Malignant. Variable appearances (epithelioid to spindled). Large nuclei, prominent
nucleoli. Absence of maturation. Lots of mitoses. Extensive pagetoid spread.
Basal Cell Carcinoma—Like elsewhere on the skin. Basaloid cells with peripheral palisading.
Bartholin Gland Carcinomas—can be SCC, adenocarcinomas, transitional cell, etc…
Mammary-type Adenocarcinoma—like breast cancers in the breast, thought to arise from anogenital
mammary-like glands. Notably, you can get phyllodes tumors too!
Adenocarcinoma of Skene glands—resembles prostate cancer. Stains with PSA
Unique Vulvar Mesenchymal Lesions
Fibroepithelial Stromal Polyp
Benign.
Polypoid growth with variably cellular central
fibrovascular core covered in squamous epithelium.
Stroma contains predominantly bland spindled cells. Can
see multinucleated stroma cells with degenerative-type
atypia including significant pleomorphism.
Most common in reproductive age women.
Can grow during pregnancy.
Aggressive Angiomyxoma
Benign (despite name!), but with a tendency to
recur after incomplete recurrence.
Often presents as a “cyst” in reproductive age
Large (>5 cm), poorly-circumscribed, infiltrative.
Gelatinous consistency.
Low-grade, hypocellular. Composed of small,
bland spindled cells with scant cytoplasm.
Numerous blood vessels of varying sizes,
including thin-walled capillary-like and thick-
walled arteries with radiating perivascular
smooth muscle.
Invades fat and muscle. Extravasated RBCs.
No mitotic activity of atypia.
IHC: (+)ER, PR, desmin. (+/-)CD34
Molecular: HMGA2 rearrangements
Treatment: Complete surgical resection. Most
people treated with first surgery.
Superficial Angiomyxoma
Benign with localized recurrences.
Small (<5 cm), exophytic polypoid mass centered in skin
and subcutaneous tissue (“Superficial”!!). Multilobulated.
Well-dermarcated, but unencapsulated.
Hypocellular myxoid nodules in dermis.
Bland stellate and spindled cells and inflammatory cells
(classically neutrophils) and numerous delicate vessels.
Can envelope skin adnexal structures/epithelium
Cellular Angiofibroma
Benign. Usually painless superficial mass or polyp.
Small (<5 cm). Rare.
Circumscribed, but unencapsulated. Often traps fat at edges.
Composed of uniform bland spindled cells in fibrous stroma.
Small to medium-sized blood vessels with thick hyalinized
walls.
Sort of resembles a spindle-cell lipoma, but with wispy
collagen.
Superficial Myofibroblastoma
Benign.
Discrete, unencapsulated. Usually small (< 5 cm)
Oval to spindled cells with wavy nuclei and scant
cytoplasm
Fine collagenous stroma. Varied architecture.
Thin-walled vessels, which might be dilated and
“Stag-horn”
IHC: (+) Desmin, ER/PR; (+/-) CD34
Angiomyofibroblastoma
Benign. Non-recurring.
Small (<5 cm), circumscribed.
Alternating hypocellular and
hypercellular areas
Spindle and plump epithelioid or
plasmacytoid cells