CARCINOMA VULVA

•Mons pubis •Labia majora•Labia minora•Clitoris •Vestibule•Perineum•Bartholin gland•Vestibular

gland

Vulva - AnatomyVulva - Anatomy

Incidence Incidence

• 4-5% of the genital tract malignancies

• Post-menopausal women

• Highest between 60-70 years

ETIOLOGY ETIOLOGY

Still unknown, but certain Still unknown, but certain associatedassociated

etiological factors:etiological factors:

• Human Papilloma virus Human Papilloma virus ( 16,18,31)( 16,18,31)

• Herpes Simplex Type II virusHerpes Simplex Type II virus

• Carcinoma cervix (20-50%)Carcinoma cervix (20-50%)

• Immune suppressionImmune suppression

ETIOLOGYETIOLOGY

• Lower socioeconomic classLower socioeconomic class• Poor hygienePoor hygiene• SmokingSmoking• Chronic granulomatous venereal Chronic granulomatous venereal

lesionslesions• Diabetes MellitusDiabetes Mellitus• Hypertension & arteriosclerosisHypertension & arteriosclerosis• ObesityObesity

Contd.

Lichen sclerosis

pre-cancerous

•Asymptomatic 20%

•Pruritus vulvae 71%

•Vulvar mass 58%

•Ulceration 28%

•Bleeding 26%

•Vulvar pain 23%

•Urinary tract symptoms 14%

•Discharge 13%

CLINICAL FEATURES

SITESITE

• Labia Majora Labia Majora 75%75%

• ClitorisClitoris

• Labia MinoraLabia Minora

• Posterior FourchettePosterior Fourchette

Less common

Advanced squamous cell carcinoma

SITESITE

ROUTES OF SPREADROUTES OF SPREAD

LocalLocal • Vagina Vagina • PerineumPerineum• Anal canalAnal canal• Urethra Urethra • BoneBone

LymphaticLymphatic

•Superficial Superficial

inguinalinguinal

•Deep inguinalDeep inguinal

•Pelvic nodesPelvic nodes

Lymphatic drainage

PATHOLOGYPATHOLOGY

Squamous cellSquamous cell 90%90%MelanomaMelanoma 5-10%5-10%Basal cell Basal cell carcinomacarcinoma

2%2%

AdenocarcinomaAdenocarcinoma 1%1%

FIGO staging (1995)

Stage I Tumour confined to the vulva or perineum , 2 cm or < in greater dimension. No nodal metastasis

Stage Ia

Less than 1mm stromal invasion

Stage Ib

>1 mm stromal invasion

Stage II Tumour confined to the vulva or perineum or both more than 2 cm in greatest dimension. No nodal metastasis

Stage III Extends beyond the vulva, vagina, lower urethra or anus; or unilateral regional lymph node metastasis

Stage IVa

Involves the mucosa of rectum or bladder; upper urethra; or pelvic bone; and / or bilateral regional lymph node metastasis

Stage IVb

Any distant metastasis, including pelvic lymph node

FIGO staging (1995)

DIAGNOSISDIAGNOSIS

•HistoryHistory

•ExaminationExamination

•BiopsyBiopsy

Histological ClassificationHistological Classification

Squamous carcinoma in situ (VIN III) Squamous carcinoma in situ (VIN III)        • Paget's disease Paget's disease        • InvasiveInvasive

– Squamous cell carcinoma Squamous cell carcinoma  – Basal cell Basal cell  – Malignant melanoma Malignant melanoma  – Adenocarcinoma (Bartholin's gland or Skene's Adenocarcinoma (Bartholin's gland or Skene's

gland)gland)

VIN Invasive VIN

Pathology

Basal and Squamous cell ca

Squamous cell carcinoma

Pathology

Histopathology

VIN

Histopathology

Histopathology

Paget’s disease

MANAGEMENTMANAGEMENT

MAINSTAY OF TREATMENT IS MAINSTAY OF TREATMENT IS SURGERYSURGERY

• HEMIVULVECTOMYHEMIVULVECTOMY

• RADICAL VULVECTOMY WITH RADICAL VULVECTOMY WITH LYMPHADENECTOMYLYMPHADENECTOMY EN BLOC DISSECTION BY BUTTERFLY EN BLOC DISSECTION BY BUTTERFLY

INCISIONINCISION SEPARATE VULVAR & INGUINAL INCISIONS SEPARATE VULVAR & INGUINAL INCISIONS

(TRIPLE INCISION)(TRIPLE INCISION)

PREOPERATIVE PREOPERATIVE EVALUATIONEVALUATION

• Blood CPBlood CP• Blood glucose random Blood glucose random • Urine REUrine RE• X-ray chest, ECGX-ray chest, ECG• Pap’s smearPap’s smear• Pelvic ultrasoundPelvic ultrasound

IN ADVANCED CASESIN ADVANCED CASES• Colposcopy, Cystoscopy, ProctoscopyColposcopy, Cystoscopy, Proctoscopy CT Scan, MRICT Scan, MRI

COMPLICATIONS OF COMPLICATIONS OF SURGERYSURGERY

IMMEDIATEIMMEDIATE

• HaemorrhageHaemorrhage

• Wound induration Wound induration

• Partial wound dehiscencePartial wound dehiscence

• Infection Infection

LATE COMPLICATIONSLATE COMPLICATIONS

•Lymphoedema

•Osteitis pubis

•Sexual dysfunction

•Femoral inguinal hernia

•DVTDVT

•ParaesthesiaParaesthesia

ADJUVANT ADJUVANT RADIOTHERAPHYRADIOTHERAPHY

•Pre operativePre operative

•Post operativePost operative

CHEMORADIOTHERAPCHEMORADIOTHERAPHYHY

Has a role in clitoris & Has a role in clitoris & sphincter preserving surgery in sphincter preserving surgery in advanced casesadvanced cases

Follow UpFollow UpPost SurgicalPost Surgical• Every 4 months Every 4 months 1 Year 1 Year • Every 6 monthsEvery 6 months 2 – 5 Years2 – 5 Years• Annually Annually + 5 Years+ 5 Years

Post Radiation Post Radiation • First visit First visit 1 Month1 Month• Every 2 monthsEvery 2 months 1 Year1 Year• Every 6 monthsEvery 6 months 2 – 3 Years 2 – 3 Years • Annually Annually + 3 Years+ 3 Years

SURVIVAL RESULTSSURVIVAL RESULTSDepend on the extent of the Depend on the extent of the disease at the time of diagnosis disease at the time of diagnosis and treatment undertakenand treatment undertaken

Five year survival rate.Five year survival rate.

1.1. Stage I & II Stage I & II 90%90%

2.2. All stagesAll stages 75%75%

3.3. Negative lymph nodes Negative lymph nodes 96%96%

4.4. Positive lymph nodes Positive lymph nodes 66%66%