Vulvar and Vaginal Vulvar and Vaginal lesionslesions

Dr.F Behnamfar MDDr.F Behnamfar MD

Introduction Most usful means of generating

differential diagnosis is by morphological findings rather than symptomatology

Vulvar biopsy should be performed if the lesion is clinically suspicious or does not resolve after standard therapy

Vulvar SymptomsVulvar Symptoms Most often,primary vaginitis and Most often,primary vaginitis and

secondary vulvitissecondary vulvitis

A number of skin conditions on A number of skin conditions on other areas of the bodyother areas of the body

Neoplasia Vulvar intraepithelial neoplasia a precancerous lesion that may progress to

invasive cancer

Most are raised multifocal white (may be red or pink) and/or verrucous lesions

Cancer presents with unifocal vulvar plaque,ulcer or mass

Lichen scerosus and erosive lichen planus predispose to cancer

Genital warts Caused by human papillomavirus Flat,filliform or verrucous,or giant Flesh colored or pigmented Biopsy is indicated if there is rapid

growth,increased pigmentation,ulceration,pigmentation,fixation or poor response to therapy

Treatment : trichloroacetic acid, podophyllum,Cryo,laser

Not curative ,merely speed clinical resolution

White patch Lichen sclerosus,well demarcated

white finely wrinkled and atrophic patches

Vulvar itching and typical findings Potent topical corticosteriod

ointment Close follow up for risk of

malignancy

Other vulvar Other vulvar conditionsconditions folliculitisfolliculitis

Fox.fordiyce diseaseFox.fordiyce disease

Acanthosis nigricansAcanthosis nigricans

Extramammary pagets Extramammary pagets disease,intraepithelial adenocarcinomadisease,intraepithelial adenocarcinoma

Herpes simplex Scabis

Vulvar cysts, tumors Vulvar cysts, tumors and massesand masses Condylomata accuminataCondylomata accuminata

duct cysts,Skenes duct cystsduct cysts,Skenes duct cysts

Vulvar Ulcers: Behcet Vulvar Ulcers: Behcet disease,lichen planusdisease,lichen planus

Vaginal ConditionsVaginal Conditions Retained foreign bodyRetained foreign body UlcerationUlceration MalignancyMalignancy

Vulvar CancerVulvar Cancer 3870 new cases 20053870 new cases 2005 870 deaths870 deaths Approximately 5% of Gynecologic Approximately 5% of Gynecologic

CancersCancers

American Cancer Society. Cancer Facts & Figures. 2004. Atlanta, GA; 2005

Vulvar CancerVulvar Cancer 85% Squamous Cell Carcinoma85% Squamous Cell Carcinoma 5% Melanoma5% Melanoma 2% Sarcoma2% Sarcoma 8% Others8% Others

Vulvar CancerVulvar Cancer Biphasic Distribution ,two distinct Biphasic Distribution ,two distinct

etiologies:etiologies:– Age 70 Age 70 – type, unifocal,type, unifocal,– in areas adjacent to lichen sclerosus or squamous in areas adjacent to lichen sclerosus or squamous

hyperplasia (Chronic inflammatory conditions)hyperplasia (Chronic inflammatory conditions)

– 20% in patients UNDER 40 and appears to be 20% in patients UNDER 40 and appears to be increasing,increasing,

– multifocal,multifocal,– basaloid or warty types,basaloid or warty types,– HPV related,smoking and VIN HPV related,smoking and VIN

Vulvar CancerVulvar Cancer Paget’s Disease of VulvaPaget’s Disease of Vulva

– 10% will be invasive10% will be invasive– 4-8% association with underlying 4-8% association with underlying

Adenocarcinoma of the vulvaAdenocarcinoma of the vulva

SymptomsSymptoms Most patients are treated for Most patients are treated for

“other” conditions“other” conditions 12 month or greater time from 12 month or greater time from

symptoms to diagnosissymptoms to diagnosis

SymptomsSymptoms PruritusPruritus MassMass PainPain BleedingBleeding UlcerationUlceration DysuriaDysuria DischargeDischarge Groin MassGroin Mass

SymptomsSymptoms May look like:May look like:

– RaisedRaised– ErythematousErythematous– UlceratedUlcerated– CondylomatousCondylomatous– NodularNodular

Vulvar CancerVulvar Cancer IF IT LOOKS ABNORMAL ON THE IF IT LOOKS ABNORMAL ON THE

VULVAVULVA BIOPSY!BIOPSY! BIOPSY!BIOPSY! BIOPSY!BIOPSY!

Tumor SpreadTumor Spread Very Specific nodal spread Very Specific nodal spread

patternpattern Direct SpreadDirect Spread HematogenousHematogenous

StagingStaging Based on TNM Surgical Staging Based on TNM Surgical Staging

– Tumor sizeTumor size– Node StatusNode Status– Metastatic DiseaseMetastatic Disease

StagingStaging Stage I T1 N0 M0Stage I T1 N0 M0

– Tumor ≤ 2cm Tumor ≤ 2cm

– IAIA ≤1 mm depth of stromal ≤1 mm depth of stromal InvasionInvasion

– IBIB 1 mm or more depth of 1 mm or more depth of invasioninvasion

StagingStaging Stage II T2 N0 M0Stage II T2 N0 M0

– Tumor >2 cmTumor >2 cm– Confined to Vulva or PerineumConfined to Vulva or Perineum

StagingStaging Stage IIIStage III

– T3 N0 M0T3 N0 M0– T3 N1 M0T3 N1 M0– T1 N1 M0T1 N1 M0– T2 N1 M0T2 N1 M0

Tumor any size involving lower urethra, Tumor any size involving lower urethra, vagina, anus OR unilateral positive vagina, anus OR unilateral positive nodesnodes

StagingStaging Stage IVAStage IVA

– T1 N2 M0T1 N2 M0– T2 N2 M0T2 N2 M0– T3 N2 M0T3 N2 M0– T4 N any M0T4 N any M0

Tumor invading upper urethra, bladder, Tumor invading upper urethra, bladder, rectum, pelvic bone or bilateral nodesrectum, pelvic bone or bilateral nodes

StagingStaging Stage IVBStage IVB

– Any T Any N M1Any T Any N M1 Any distal mets including pelvic nodesAny distal mets including pelvic nodes

TreatmentTreatment Primarily SurgicalPrimarily Surgical

– Wide Local ExcisionWide Local Excision– Radical ExcisionRadical Excision– Radical Vulvectomy with Inguinal Radical Vulvectomy with Inguinal

Node DissectionNode Dissection UnilateralUnilateral BilateralBilateral Possible Node Mapping, still Possible Node Mapping, still

investigationalinvestigational

TreatmentTreatment Local advanced may be treated Local advanced may be treated

with Radiation plus with Radiation plus ChemosensitizerChemosensitizer

Positive Nodal StatusPositive Nodal Status– 1 or 2 microscopic nodes < 5mm 1 or 2 microscopic nodes < 5mm

can be observedcan be observed– 3 or more or >5mm post op 3 or more or >5mm post op

radiationradiation

New advances in New advances in treatmenttreatment Individualization of treatment,vulvar Individualization of treatment,vulvar

conservation for unifocal tumorsconservation for unifocal tumors Elimination of routine pelvic Elimination of routine pelvic

lymphadenectomy lymphadenectomy Omission of groin dissection for T1 Omission of groin dissection for T1

tumors (<1mm stromal invasion)tumors (<1mm stromal invasion) Separate incisions improve wound Separate incisions improve wound

healinghealing

TreatmentTreatment Special TumorSpecial Tumor

– Verrucous CarcinomaVerrucous Carcinoma Indolent tumor with local disease, rare Indolent tumor with local disease, rare

mets UNLESS given radiation, becomes mets UNLESS given radiation, becomes Highly malignant and aggressiveHighly malignant and aggressive

Excision or Vulvectomy ONLYExcision or Vulvectomy ONLY

Vulva 5 year survivalVulva 5 year survival Stage IStage I 9090 Stage IIStage II 7777 Stage IIIStage III 5151 Stage IVStage IV 1818

Hacker and Berek, Practical Gynecologic Oncology 4th Edition, 2005

RecurrenceRecurrence Local Recurrence in VulvaLocal Recurrence in Vulva

– Reexcision or radiation and good Reexcision or radiation and good prognosis if not in original site of prognosis if not in original site of tumortumor

– Poor prognosis if in original sitePoor prognosis if in original site

RecurrenceRecurrence Distal or MetastaticDistal or Metastatic

– Very poor prognosis, active agents Very poor prognosis, active agents include Cisplatin, mitomycin C, include Cisplatin, mitomycin C, bleomycin, methotrexate and bleomycin, methotrexate and cyclophosphamide cyclophosphamide

MelanomaMelanoma 5% of Vulvar Cancers5% of Vulvar Cancers Not UV relatedNot UV related Commonly periclitoral or labia Commonly periclitoral or labia

minoraminora

MelanomaMelanoma Microstaged by one of 3 criteriaMicrostaged by one of 3 criteria

– Clark’s LevelClark’s Level– Chung’s LevelChung’s Level– BreslowBreslow

Melanoma TreatmentMelanoma Treatment Wide local or Wide Radical Wide local or Wide Radical

excision with bilateral groin excision with bilateral groin dissectiondissection

Interferon Alpha 2-bInterferon Alpha 2-b

Vaginal CarcinomaVaginal Carcinoma 2140 new cases projected 20052140 new cases projected 2005

810 deaths projected 2005810 deaths projected 2005 Represents 2-3% of Pelvic Represents 2-3% of Pelvic

CancersCancers

American Cancer Society. Cancer Facts & Figures. 2004. Atlanta, GA; 2005

Vaginal CancerVaginal Cancer 84% of cancers in vaginal area 84% of cancers in vaginal area

are secondaryare secondary– CervicalCervical– UterineUterine– ColorectalColorectal– OvaryOvary– VaginaVagina

Fu YS, Pathology of the Uterine Cervix, Vagina and Vulva, 2nd ed. 2002

Vaginal CarcinomaVaginal Carcinoma Squamous CellSquamous Cell 80-85%80-85% Clear CellClear Cell 10%10% SarcomaSarcoma 3-4%3-4% MelanomaMelanoma 2-3%2-3%

Clear Cell CarcinomaClear Cell Carcinoma Associated with DES Exposure In Associated with DES Exposure In

UteroUtero– DES used as anti abortifcant from DES used as anti abortifcant from

1949-19711949-1971– 500+ cases confirmed by DES 500+ cases confirmed by DES

RegistryRegistry– Usually occurred late teensUsually occurred late teens

Vaginal Cancer Vaginal Cancer EtiologyEtiology Mimics Cervical CarcinomaMimics Cervical Carcinoma

– HPV 16 and 18HPV 16 and 18

StagingStaging Stage IStage I Confined to Vaginal WallConfined to Vaginal Wall Stage IIStage II Subvaginal tissue but not Subvaginal tissue but not

to pelvic sidewallto pelvic sidewall Stage IIIStage III Extended to pelvic Extended to pelvic

sidewallsidewall Stage IVAStage IVA Bowel or BladderBowel or Bladder Stage IVBStage IVB Distant metsDistant mets

TreatmentTreatment Surgery with Radical Surgery with Radical

Hysterectomy and pelvic lymph Hysterectomy and pelvic lymph dissection in selected stage I dissection in selected stage I tumors high in Vaginatumors high in Vagina

All others treated with radiation All others treated with radiation with chemosensitizationwith chemosensitization

5 year Survival5 year Survival Stage IStage I 70%70% Stage IIStage II 51%51% Stage IIIStage III 33%33% Stage IVStage IV 17%17%