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Vulva

Reviewer: Monika Roychowdhury, M.D. (see Reviewers page)
Revised: 29 March 2013, last major update IN PROGRESS
Copyright: (c) 2002-2013, PathologyOutlines.com, Inc.

Table of contents

General: primary references   embryology   normal anatomy   ectopic mammary tissue   inflammatory lesions   infection

Non-neoplastic disorders: Bartholin’s cyst   Behcet’s disease   leukoplakia   lichenification   lichen planus   lichen sclerosus   multinucleated atypia   squamous cell hyperplasia   vulvar dystrophy   vulvar hypertrophy with lymphedema

Benign tumors: angiomyofibroblastoma   benign skin adnexal tumors   bowenoid papulosis   cellular angiofibroma   fibroepithelial polyp   genital nevus   hidradenoma papilliferum   hypertrophic HSV   leiomyoma   melanocytic nevi   mixed tumor   mucosal polyp   pseudosarcomatous fibroepithelial stromal polyps   squamous papilloma   vulvar vestibular papillomatosis

Premalignant lesions: atypical melanocytic nevi of genital type   condyloma   differentiated vulvar intraepithelial neoplasia   dysplastic nevi   vulvar intraepithelial neoplasia

Malignant neoplasms: aggressive angiomyxoma   Bartholin’s gland carcinoma   basal cell carcinoma   basaloid carcinoma   Bowen’s disease   carcinoma-general   case reports   epithelioid sarcoma   leiomyosarcoma   melanoma   Merkel cell carcinoma   metastases   Paget’s disease   pigmented lesions   squamous cell carcinoma   verrucous carcinoma   warty carcinoma

Miscellaneous: staging   features to report


Primary references:
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AJCC Cancer Staging Manual (7th ed)

Non-neoplastic disorders
Multinucleated atypia

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Usually young women

Micro: multinucleated epithelioid cells in lower to middle epithelial layers, containing 2-10 nuclei, often prominent nucleoli; no surface atypia, no hyperchromasia or pleomorphism; no significant inflammation

Negative stains: HSV

Molecular: HPV negative

Reference: AJSP 1994;18:1233

 

Squamous cell hyperplasia (keratosis)

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Associated with cancer, but not considered premalignant unless VIN present

Do not appear to be precursor to HIV negative squamous cell carcinoma, Hum Path 1996;27:389

Suggested to diagnose as dermatosis, not a squamous hyperplasia, Mod Path 1997;10:401

Micro: epithelial hyperplasia and hyperkeratosis; increased mitotic figures in basal and prickle cell layers; mild dermal chronic inflammation; no atypia

 

Vulvar dystrophy

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Unrelated disorders that present as irregular patchy areas of thickened skin and severe itching

Often white skin (leukoplakia), but may be red

Easily traumatized

Suggested to diagnose as dermatosis, not as vulvar dystrophy, Mod Path 1997;10:401

Kraurosis: accompanied by soft tissue atrophy

Mixed vulvar dystrophy: squamous hyperplasia and lichen sclerosus

 

Vulvar hypertrophy with lymphedema

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Case report of 43 year old quadriplegic woman with bilateral vulvar enlargement, Archives 2000;124:1697

Micro: hypocellular proliferation of fibroblastic cells in edematous-myxoid stroma, vessels with perivascular collagen deposition, ectatic tortuous lymphatics; no true thick-walled vessels

Micro images: image1, image2, image3

DD: aggressive angiomyxoma

 

Case reports

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Angiokeratoma of clitoris, Archives 1992;116:880

Desmoid tumor associated with pregnancy, Archives 1997;121:512

Extrapulmonary sugar tumor, Mod Path 2001;14:615

Granular cell tumor of clitoris, AJSP 1986;10:497

Lymphoma, T cell, in renal allograft recipient, AJSP 1993;17:842

Malakoplakia of Bartholin’s glands, Archives 1986;110:757

Neurofibromatosis, AJSP 1989;13:873

Phyllodes tumor, recurrent, in aberrant breast tissue, AJSP 1993;17:946

Solitary fibrous tumors, Mod Path 1997;10:1028

Sweat gland carcinomas, Archives 1985;109:43

Synovial sarcoma, Mod Path 1996;9:970

Undifferentiated sweat gland carcinoma mimicking epithelioid sarcoma, AJSP 1991;15:990

 

Benign or non-neoplastic tumors

Angiomyofibroblastoma

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Benign vulvar tumor, first described in 1992, AJSP 1992;16:373

Case reports: 30 year old woman with 2 cm circumscribed mass (Case of Week #241); 80 year old woman with sarcomatous transformation (Am J Surg Pathol 1997;21:1104)

No reported recurrences after excision

Gross: well circumscribed, 0.5 to 12.0 cm

Micro: circumscribed tumor with alternating hypercellular and hypocellular areas, spindle cells and plump stroma cells with eosinophilic cytoplasm that aggregate around small blood vessels; may have binucleated or multinucleated cells; minimal stromal mucin, rare/no red blood cell extravasation, no atypia; rare/no mitotic figures; mast cells common

Positive stains: vimentin, desmin

Negative stains: actin, keratin, S100

EM: well developed rough endoplasmic reticulum, Golgi apparatus, intermediate filaments, pinocytotic vesicles

DD: angiomyxoma (not circumscribed, less cellular, fewer vessels, no hyalinized vessels, infrequent plump stromal cells, stromal mucin, RBC extravasation)

Reference: Mod Path 1996;9:284

 

Benign skin adnexal tumors

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Benign pilar tumor, syringoma, warty dyskeratoma

 

Bowenoid papulosis

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Type of vulvar intraepithelial neoplasia

Multiple small papules, often pigmented, in vulva of young women

Grossly resemble verrucae

Does not progress to invasive carcinoma, but may recur

Distinction between Bowen’s disease/CIS and bowenoid papulosis can usually be made clinically

Micro: atypia up to carcinoma in-situ, non-invasive; dysplastic cells arise in background of orderly epithelium with sparing of acrotrichium

Positive stains: HPV, p53

 

Cellular angiofibroma

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Women ages 39-50 years

Resemble labial or Bartholin’s gland cyst

First described in 1997, benign behavior in 2 of 2 cases

Gross: usually < 3 cm, well circumscribed

Micro: cellular neoplasm composed of uniform, bland, spindled cells, numerous thick walled and often hyalinized vessels, rare adipocytes; brisk mitotic activity

Positive stains: vimentin

Negative stains: CD34, S100, actin, desmin, EMA

DD: aggressive angiomyxoma, angiomyofibroblastoma, spindle cell lipoma, solitary fibrous tumor, perineuroma, leiomyoma

Reference: AJSP 1997;21:636

 

Fibroepithelial polyp

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Loose myxoid stroma covered by squamous epithelium

May have bizarre, multinucleated stromal cells

Positive stains: desmin (often)

 

Genital nevus

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Prominent junctional nests with large cells

Seen in young women;

May be overdiagnosed as melanoma (usually age 50+)

 

Hidradenoma papilliferum

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Aka papillary hidradenoma

Benign, small, sharply circumscribed nodule covered by normal skin, often on labia majora or interlabial folds

May ulcerate through skin and simulate carcinoma

May derive from ectopic mammary tissue (resembles intraductal papilloma)

Case report with associated intraductal apocrine breast cancer (GCDFP-15+, ER+, PR+), Archives 1991;115:1249

Micro: well differentiated, complex papillary glandular pattern with some stratification and pleomorphism; myoepithelial layer usually present

 

Hypertrophic herpes simplex virus in HIV positive patient

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Rare; chronic hyperproliferative plaque or mass that clinically resembles malignancy

In vulva or perianal region

Case reports: Case of Week #53, 44 year-old African American man with a hypertrophic gluteal cleft plaque (J Drugs Dermatol 2003;2:198, free full text)

Treatment: often ineffective, often recurs

Gross images: vulvar mass

Micro: epithelial hyperplasia, brisk infiltrate of lymphocytes and plasma cells

Micro images: vulvar mass #1; #2; HSV immunostain

Positive stains: HSV

References: Dis Colon Rectum 2005;48:2289, BHIVA Conf 2005 Apr 20-23;11:PA8

 

Leiomyoma

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Diagnosis: only 1 of these criteria (if 2, atypical leiomyoma; if 3, leiomyosarcoma): 5 cm+, infiltrative markers, 5+ mitotic figures/10 HPF, moderate/severe cytologic atypia, AJSP 1996;20:779

Treatment: conservative excision with long-term, careful follow up

Positive stains: ER, PR

 

Melanocytic nevi

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Common in labia majora, usually intradermal or compound in adults

 

Mixed tumor

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Aka chondroid syringoma

Benign, but looks different from mixed tumors elsewhere

 

Mucosal polyp

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Benign stroma covered with squamous epithelium

 

Pseudosarcomatous fibroepithelial stromal polyps

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Resemble fibroepithelial stromal polyps of vagina, cervix and vulva, but with bizarre morphology, frequent mitoses (>10/10 HPF), atypical mitotic figures or hypercellularity

Median age 32, range 16-75 years

Often in pregnant patients

May recur locally; no metastases

Positive margin status, which is common, apparently not associated with recurrence

Gross: often multiple lesions, particularly in pregnant women

Positive stains: desmin

DD: aggressive angiomyxoma: deep, prominent vascular pattern cuffed by myoid bundles

angiomyofibroblastoma: well circumscribed subserosal nodule, no atypia, stromal cells cluster around vessels, which usually have delicate walls

botyroid embryonal rhabdomyosarcoma: early childhood, submucosal hypercellular zone/cambium layer, rhabdomyoblasts, myoglobin+, myogenin+

cellular angiofibroma: well circumscribed, less polypoid, diffusely vascular with hyalinized walls, no atypical stromal cells, desmin-

DFSP: CD34+, desmin-, storiform throughout

leiomyosarcoma: clear boundary of tumor cells with epithelium, smooth muscle differentiation

low grade endometrial stromal sarcoma: vessels resemble spiral arterioles, no central vascular core, thick bands of collagen in starburst pattern, dot like staining of desmin or keratin

MPNST: perivascular accentuation, 50% are S100+

Reference: AJSP 2000;24:231

 

Squamous papilloma

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Papillary vulvar lesions without atypia

May contain HPV by PCR
No microscopic features of condyloma

 

Vulvar vestibular papillomatosis

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Lesions resemble condyloma but most are HPV negative

May be normal anatomic variant

Micro: like fingers, not like stalks of condylomas

 

Premalignant lesions

Atypical melanocytic nevi of the genital type

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Usually on vulva (labia minora or clitoral region), also perineum, rarely mons pubis, axilla, male genitalia

Median age 25 years

Micro: nonspecific stromal pattern

DD: melanoma

Reference: Hum Path 1998;29 (1 Suppl 1):S1

 

Condyloma

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Sexual transmitted disease, lesions may be multiple and coalesce

Vulvar, vaginal, perianal, perineal

Different from condyloma latum of syphilis

Flat condyloma more common than condyloma accuminatum

MIB staining present in 2+ epithelial cells in upper 2/3 of epithelium anywhere within the lesion, AJSP 2000;24:1393

Regress, except in immunocompromised

Treatment: CO2 laser, podophyllin application (for one week, causes epidermal pallor, keratinocyte necrosis, increased mitotic figures)

Micro: complex papillary arrangement of well differentiated squamous cells with intact basement membranes, well vascularized connective tissue stalk or flat lesion; T lymphocytes; acanthosis, hyperkeratosis, parakeratosis; orderly maturation but koilocytotic atypia (nuclear hyperchromasia, enlargement, pleomorphism and cytoplasmic cavitation), no other significant atypia, no atypical mitotic figures

May have pseudobowenoid changes resembling podophyllin effect or VIN (superficial keratinocytes in various stages of apoptosis, nuclear degenerative changes of absence of nuclear membrane and dispersed chromatin), Mod Path 1996;9:375

Molecular: diploid, tetraploid, octaploid

DD: vulvar intraepithelial neoplasia (abnormal mitotic figures, lack of maturation, nuclear pleomorphism, enlargement and hyperchromasia), squamous papillomas (no atypia)

 

Differentiated vulvar intraepithelial neoplasia

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Maturation, variable hyperplasia, keratinization and parabasal atypia

Variable HPV

 

Dysplastic nevi

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More common on labia majora than labia minora

Micro: stroma has concentric eosinophilic fibroplasia or lamellar fibroplasia, Hum Path 1998;29(1 Suppl 1):S1

 

Vulvar intraepithelial neoplasia (VIN)

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Premalignant lesion

Incidence has markedly increased recently; becoming more common in women 20 to 35 years

Frequently coexists with HPV infection

Multifocal

VIN1 to VIN3 (carcinoma in situ / Bowen’s disease)

 

Malignant neoplasms

Aggressive angiomyxoma

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Bland appearing, slow growing, perineal lesions that may present at vulvar mass and simulate a Bartholin’s gland cyst

Usually teens/young adults, rarely children; occurs in men in scrotum

Recurs locally in ischiorectal or retroperitoneal space, perhaps due to incomplete excision, Hum Path 1985;16:621

Treatment: wide local excision

Case Reports: 28 year old woman with vulvar tumor (Case of the Week #181)

Gross: gelatinous, ill defined mass, larger and deeper than fibroepithelial polyp

Micro: spindled and stellate cells in hypocellular myxomatous stroma, no/rare mitotic figures, no atypia; thick walled vessels with dilated lumina and occasional hyaline thickening of adventitia; extravasation of red blood cells common; no bizarre stromal cells; often deep, prominent vascular pattern cuffed by myoid bundles

Positive stains: actin

Negative stains: S100, factor 8, CEA, keratin

EM: primitive mesenchymal cells with fibroblastic features

DD: vulvar hypertrophy with lymphedema

 

Basal cell carcinoma

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Nodular masses in labia majora in elderly, may be large and ulcerate

Solid, keratotic, adenoid types

Note: basal cell carcinoma may exhibit abrupt squamous differentiation of pilar type, but doesn’t affect prognosis

Rare nodal metastases seen with deeply invasive lesions

DD: basaloid carcinoma, basaloid changes in Bowen’s disease, invasive squamous cell carcinoma

 

Basaloid carcinoma

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Micro: relatively uniform population of small, ovoid cells with high N/C ratio resembling VIN 3; peripheral pallisading, resembles counterpart in upper aerodigestive tract; may have adenoid cystic component; associated with VIN

Molecular: HPV positive

Reference: AJSP 1993;17:133

 

Bowen’s disease

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Aka carcinoma in situ; a type of vulvar intraepithelial neoplasia

10% progress to invasive carcinoma if untreated

Treatment: wide local excision if localized; tumor at margin predicts recurrence

Gross: slightly elevated, plaque-like, red velvety appearance, centered on labia majora, may extend to perineum and anus

Micro: full thickness disorganization of epithelium with hyperkeratosis, parakeratosis, acanthosis, multinucleated dyskeratotic cells, abnormal mitotic figures throughout full thickness; has intracellular bridges; often involves intraepidermal portion of hair follicle (acrotrichium), but spares counterpart in sweat gland (acrosyringium)

Positive stains: p53

Molecular: often aneuploid; HPV-16 positive

 

Carcinoma -general

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95% squamous cell carcinoma; also basal cell, melanoma, adenocarcinoma

HPV related (90% are HPV 16, 18)

Multicentric, associated with VIN III (CIS/Bowen’s disease)

May regress or progress; progression more common in elderly and immunocompromised

Carcinomas associated with lichen sclerosus or squamous hyperplasia are usually HPV-, p53+ and have poor prognosis

Tumors with prominent keratinization are often confused with dermatitis or eczema

Risk of metastases is related to size, depth, angiolymphatic invasion

Survival: 60% if 2 cm in diameter or less after vulvectomy and lymphadenectomy; <10% with positive lymph nodes or larger tumors

 

Epithelioid sarcoma

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More aggressive in vulva than in distal extremities, perhaps due to rhabdoid features at this site

Resembles malignant rhabdoid tumor

May recur locally (65%) or metastasize (75%), usually to lymph nodes, Mod Path 2001;14:655

Most die of disease (65%)

Micro: sheets of large epithelioid cells with marked cytologic atypia including prominent nucleoli, resembling poorly differentiated carcinoma, usually no granuloma-like pattern; often rhabdoid phenotype with intracytoplasmic inclusions

Micro images: image1, stains

Positive stains: vimentin, keratin, EMA (85%), p53 (80%), HMB45 (25%), CEA (10%), CD99 (25%)

Negative stains: S100, CD31

EM: inclusions in rhabdoid cells are intermediate filaments

DD: synovial sarcoma (biphasic, CD34-), angiosarcoma (CD31+, EMA-), melanoma (S100+, HMB45+), MPNST (S100+), extrarenal rhabdoid tumor, undifferentiated carcinoma (usually connection to epidermis or adnexae, squamous or glandular differentiation, CD34-)

Reference: AJSP 1997;21:130, AJSP 1989;13:848

 

Leiomyosarcoma

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Proposed criteria is 3 of these features: 5 cm or larger, infiltrative markers, 5+ mitotic figures/10 HPF, moderate/severe cytologic atypia, AJSP 1996;20:779

Treatment: wide excision, long-term careful follow-up

Positive stains: ER, PR

 

Melanoma

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Age 50+; much less frequent than squamous cell carcinoma

Associated with melanosis, Archives 1991;115:950

Most lesions are advanced (Clark’s level III, IV)

Treatment: radical vulvectomy with bilateral inguinal nodal dissection or wide local excision for small lesions with depths of 1.75 mm+

5 year survival 35% (poor prognosis for mucosal melanomas)

Prognostic factors: nodes, level / thickness

Gross: large, infiltrating, variegated; nodular areas may be less pigmented

Micro: radial growth phase melanomas have fibroplasia with a plaque like lymphocytic infiltrate and diffuse eosinophilic fibroplasias; thick or deeply invasive vertical growth phase melanomas have fibroplasia with angiogenesis or an absence of evidence for parenchymal stromal reciprocal interactions, Hum Path 1998;29(1 Suppl 1):S1

 

Clark’s levels:

Level I - in situ

Level II - extends into superficial papillary dermis

Level III - fills and expands the papillary dermis

Level IV - invades the reticular dermis

Level V - invades into fat or other deeper tissues

 

Breslow measurements:

Invasion < Invasion <0.75 mm from deep border of granular layer

Invasion 0.76 mm to 1.5 mm from deep border of granular layer

Invasion >1.5 mm from deep border of granular layer

 

Chung Modification of Breslow Measurement

1 - Intraepithelial (melanoma in situ)

2 - Invasion of < 1 mm into dermis or lamina propria

3 - Invasion 1-2 mm into subepithelial tissue

4 - Invasion > 2 mm into fibrous or fibromuscular tissue

5 - Extension into subcutaneous fat

 

Merkel cell carcinoma

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Very aggressive

May be associated with Bowen’s disease

 

Metastases to vulva

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50% from cervix; also endometrium, kidney, gastrointestinal tract

 

Paget’s disease

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Malignant glandular tumor of vulva, either primary (vulva only, a sweat gland carcinoma arising from acrosyringium or carcinoma of basal layer differentiating along eccrine lines) or secondary (due to adenocarcinoma of anorectum, urothelium, prostate)

Crusting, weeping, oozing lesion; may be erythematous

Recurrences common due to residual disease

Only rarely causes death

In vulva and perianal region, lesion is red, crusted, sharply demarcated, usually on labia majora (also male perineum)

Good prognosis if no invasive disease present since does not metastasize, although may recur as invasive disease

May be associated with VIN

May recur in split thickness skin graft

Note: in breast, presence of Paget’s cells almost always indicates an underlying carcinoma, and intradermal cells are usually mucin negative

In vulva, 70-85% of cases are primary (no underlying carcinoma) and are usually mucin positive

Primary vulvar Paget’s is usually positive for CK7 and GCDFP-15, negative for CK20

Vulvar Paget’s secondary to anorectal carcinoma is usually positive for CK20, negative for CK7, GCDFP-15

Vulvar Paget’s secondary to urothelial carcinoma is usually positive for CK7, CK20 and uroplakins UP-III, negative for GCDFP-15, Hum Path 2002;33:545

May originate from apocrine glands (GCDFP-15 positive), aberrant embryonic cells of sweat gland lineage or ectopic cells from Bartholin’s glands (similar mucin phenotypes)

Case report of pseudoinvasive disease, Archives 1998;122:471

Rarely detected by pap smear, Archives 1988;112:941

Treatment: excise margin of normal skin and subcutaneous tissue to include all sweat glands (may not identify microscopic disease at surgery)

Micro: large pale staining tumor cells, usually in lower epidermis, in nests, glandular spaces or continuously along basement membrane; also in pilosebaceous ducts and sweat glands, tumor cells have abundant cytoplasm, large nuclei, prominent nucleoli; may see cleft between tumor cells and overlying keratinocytes; basal layer still present around outside of clusters, gland formation may be present; may have intracytoplasmic melanin, lichenoid lymphocytic infiltrates in papillary dermis; associated with fibroepithelioma-like hyperplasia (9%) or papillomatous hyperplasia (AJSP 2000;24:543); also associated with carcinoma in situ of sweat glands

May resemble invasive disease due to marked intraepithelial proliferation with deep invaginations, tangential sectioning of rete pegs, papillary dermis stroma resembling desmoplastic response, Archives 1998;122:471

Primary vulvar Paget cells tend to be large with abundant pale cytoplasm, round vesicular nuclei, prominent nucleoli, no/rare mitoses

Vulvar Paget cells secondary to urothelial carcinoma resemble high-grade urothelial cells, with anaplastic nuclear features, variable nuclear shapes, coarse irregular chromatin, infrequent/nonprominent nucleoli, high mitotic rate

Vulvar Paget cells secondary to anorectal carcinoma resemble gastrointestinal glands with stratified columnar cells, goblet cells and intraluminal necrosis

Positive stains: CEA, mucicarmine, CAM5.2, B72.3, GCDFP-15, S100 (30%), MUC1, MUC5AC, CK7, UP-III (secondary to urothelial carcinomas)

Negative stains: PAS, Alcian blue, HMB45, CK20 (usually for primary cases)

EM: glandular differentiation

DD: melanoma (has intercellular bridges), invasive carcinoma

References: AJSP 2001;25:1469 (mucin gene expression), AJSP 1984;8:43 (immunostains study), AJSP 1977;1:193

 

Pigmented lesions

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Melanoma, melanosis (lentigo simplex), VIN III (gray/blue), nevus, atypical nevomelanocytic lesions of young women (highly atypical cells which mature, good prognosis, looks like acral-lentiginous melanoma)

 

Squamous cell carcinoma

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95% of vulvar carcinomas

Mean age 60-74, rare in women younger than 30 years

Risk factors: large number of sexual partners, cigarette smoking, immunodeficiency, genital granulomatous disease, cervical carcinoma (? field effect)

Two types: HPV related (younger women, often basaloid or warty histology with VIN of similar histology) and non-HPV related (usually HPV-16, older women, usually keratinizing, associated with vulvar dystrophies)

Usually on labia majora, also labia minora, clitoris

20% metastasize to regional nodes; labial tumors to superficial inguinal nodes, clitoral tumors to deep inguinal nodes

Note: enlarged, hyperplastic nodes are common source of clinical error in assessment

Clitoral tumors are associated with lichen sclerosis, often have a prominent fibromyxoid stromal response and usually are not associated with VIN 3

Treatment: wide local excision with 1 cm margins if small, superficial (3 mm) and well differentiated; or radical vulvectomy with bilateral lymph node dissection; advanced cases have iliac lymphadenectomy, pelvic extenteration; radiation therapy also used

Prognosis: 5 year survival 50-75%; based on lymph node status (most important), tumor size, depth; extracapsular nodal spread and size of metastatic focus also important

Case report: keratinizing squamous cell carcinoma with adjacent carcinoma in situ, HPV negative, in 26 year old woman, Archives 2001;125:267

Gross/micro images: image1

Micro: usually well differentiated; tumors on clitoris may be more anaplastic; VIN often present at margins

High grade lesions may show focal glandular differentiation, but don’t call adenosquamous

Molecular: loss of 4p13, 3p, 5q; gains of 3q, 8p, Mod Path 2001;14:377; HPV +, Hum Path 1991;22:711

 

Microinvasive squamous cell carcinoma

Depth of invasion < 5 mm; however, may still need inguinal lymphadenectomy

Controversial concept

 

Verrucous carcinoma

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Aka giant condyloma of Bushki/Lowenstein

A well differentiated squamous cell carcinoma, similar to counterpart in aerodigestive tract

Note: if infiltrative or atypia present, cannot call verrucous

Very good prognosis, but may die of local invasion (metastases extremely unusual)

DO NOT RADIATE (radiation causes dedifferentiation in some)

Some call hybrid if features of verrucous and squamous carcinoma

Gross: may be huge, typically is exophytic, invades locally

Micro: "baggy pants" pattern (broad bulbous masses, pushing into underlying stroma); not infiltrative, no atypia

Molecular: HPV negative

DD: condyloma accuminatum, conventional squamous cell carcinoma (infiltrative, cytologic atypia)

 

Warty (condylomatous) carcinoma

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Usually women age 60 or less, higher percentage of black women than with keratinizing squamous cell carcinoma

Resembles condyloma microscopically

Treat with radiation

Report: depth, cleft involvement, perineural invasion; other changes (severe dysplasia, etc.)

Micro: tumor cells display marked pleomorphism, atypia, enlargement and multinucleation, associated with koilocytotic atypia in adjacent epithelium; invades via single cells or clusters of cells, NOT the broad front of verrucous carcinoma; associated with VIN

Molecular: HPV often detected

Reference: AJSP 1993;17:133

 

Staging - Vulvar carcinoma

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Includes tumors with primary site of growth in vulva only; excludes mucosal melanoma

AJCC 7th edition reflects new staging adopted by FIGO in 2008

 

Primary tumor (T) and FIGO stage in ( )

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TX: Primary tumor cannot be assessed

T0: No evidence of primary tumor

Tis: Carcinoma in situ (preinvasive carcinoma) – no longer part of FIGO classification

 

T1a (IA): Lesions 2 cm or less in size, confined to the vulva or perineum, and with stromal invasion 1.0 mm or less

T1b (IB): Lesions more than 2 cm in size OR any size with stromal invasion more than 1.0 mm, confined to the vulva or perineum

T2 (II): Tumor of any size with extension to adjacent perineal structures (lower/distal 1/3 urethra, lower/distal 1/3 vagina, anal involvement)

T3 (IVA): Tumor of any size with extension to any of the following: upper/proximal 2/3 of urethra, upper/proximal 2/3 of vagina, bladder mucosa, rectal mucosa, or fixed to pelvic bone

 

Notes:

(1) The depth of invasion is measured from the epithelial-stromal junction of the adjacent most superficial dermal papilla to the deepest point of invasion

(2) FIGO uses classification T2/T3, defined as T2 in TNM

(3) FIGO uses classification T4, defined as T3 in TNM

 

Regional lymph nodes (N) and FIGO stages in ( )

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NX: Regional lymph nodes cannot be assessed

N0: No regional lymph node metastasis

N1: One or two regional lymph nodes with the following features:

N1a (IIIA): 1 lymph node metastasis 5 mm or less

N1b (IIIA): 1 lymph node metastasis 5 mm or greater

N2 (IIIB): Regional lymph node metastasis with the following features:

N2a (IIIB): Three or more lymph node metastases each less than 5 mm

N2b (IIIB): Two or more lymph node metastases 5 mm or greater

N2c (IIIC): Lymph node metastasis with extracapsular spread

N3 (IVA): Fixed or ulcerated regional lymph node metastasis

 

Note: The site and laterality of lymph node metastases should be described

 

Distant Metastasis (M) and FIGO stages in ( )

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M0: No distant metastasis

M1 (IVB): Distant metastasis (including pelvic lymph node metastasis)

 

Stage grouping

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Stage 0: Tis N0 M0

Stage I: T1 N0 M0

Stage IA: T1a N0 M0

Stage IB: T1b N0 M0

Stage II: T2 N0 M0

Stage IIIA: T1-T2 N1a-b M0

Stage IIIB: T1-T2 N2a-b M0

Stage IIIC: T1-T2 N2c M0

Stage IVA: T1-T2 N3 M0 or T3 any N M0

Stage IVB: M1

 

Note: FIGO no longer includes Stage 0 (Tis)

 

Features of tumor to report

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Anatomic site and location

Size

Depth of invasion (squamous cell carcinoma: from epithelial-stromal junction of adjacent most superficial dermal papilla to deepest point of invasion)

Histologic type

Histologic grade

Pagetoid spread

Type of invasion (infiltrating, pushing, mixed)

Angiolymphatic invasion

Adjacent organs

Margins

Presence of precancerous lesions

Presence of vulvar dystrophies

Descriptive features (ulcers, etc.)

Lymph nodes: total, number positive, location, tumor size

Involvement or not of adjacent tissue

Reference: Archives 2000;124:51


End of Vulva chapter