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Vulvar Cancer. Kathleen M. Schmeler, M.D. Assistant Professor Department of Gynecologic Oncology. Vulvar Cancer. 4th most common gynecologic cancer in USA (following uterus, ovary and cervix) Comprises 5% of gynecologic malignancies

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vulvar cancer

Vulvar Cancer

Kathleen M. Schmeler, M.D.

Assistant Professor

Department of Gynecologic Oncology

vulvar cancer2
Vulvar Cancer

4th most common gynecologic cancer in USA (following uterus, ovary and cervix)

Comprises 5% of gynecologic malignancies

There are an estimated 3,500 new cases and 870 associated deaths per year in USA

Mean age at diagnosis is 65y, but is decreasing

vulvar cancer3
Vulvar Cancer

Risk Factors:

Cigarette smoking

Human Papilloma Virus (HPV) infection

Immunosuppression

Chronic vulvar conditions such as lichen sclerosus

VIN/CIN

Prior history of cervical cancer

vulvar cancer4
Vulvar Cancer

Two pathways of vulvar carcinogenesis:

HPV infection (60%)

Chronic inflammatory (vulvar dystrophy) or autoimmune processes

clinical manifestations
Clinical Manifestations

Most patients present with a single vulvar plaque, ulcer or mass

Labia majora is the most common site

Lesions are multifocal in 5% of cases so complete examination of the vulva, perianal area, vagina and cervix is required

A synchronous second malignancy is found in 22% of cases, usually CIN/cervical cancer

clinical manifestations12
Clinical Manifestations

Pruritus is the most common presenting symptom (especially if associated with vulvar dystrophy such as lichen sclerosus)

Vulvar bleeding or discharge

Dysuria

Enlarged groin lymph node

diagnosis
Diagnosis

Biopsy of gross lesions

If no gross lesion present but high clinical suspicion, perform colposcopy with 5% acetic acid solution

types of vulvar cancer
Types of Vulvar Cancer

Squamous cell carcinoma (>90% of cases)

Melanoma

Sarcoma

Basal cell carcinoma

Verrucous carcinoma

Adenocarcinoma (Bartholin gland)

Breast carcinoma (ectopic breast tissue in milk line that extends to perineum)

squamous cell carcinoma scca
Squamous Cell Carcinoma (SCCA)

Most common type (>90%)

Two subtypes:

1. Warty/Basaloid:

associated with HPV infection

younger women

2. Keratinizing/Simplex/Differentiated:

associated with vulvar dystrophies (e.g. lichen sclerosus)

NOT HPV related

older women

treatment of scca vulva20
Treatment of SCCA Vulva

Historically all patients were treated with radical vulvectomy and en-bloc inguinal-femoral lymphadenectomy through one incision (“Butterfly” or “Longhorn” incision)

Significant morbidity was associated with this approach, including wound breakdown, lymphedema and sexual dysfunction

Alternative surgical procedures that remove less of the vulva and surrounding skin are now used (“three-incision technique”)

treatment of scca vulva22
Treatment of SCCA Vulva

Wide Radical Excision (WRE):

Excision of vulvar lesion down to the fascia of the urogenital diaphragm

2 cm tumor-free margin

treatment of scca vulva23
Treatment of SCCA Vulva

Inguinal-Femoral Lymphadenectomy:

Removal of the superficial inguinal and deep femoral lymph nodes

Performed through separate incisions from the vulvar resection (“three-incision technique”)

Unilateral lymphadenectomy may be performed if unifocal lesion that is lateral (>2 cm from midline)

Sentinel lymph node biopsy is appropriate as an alternative to a complete inguinal-femoral lymphadenectomy in some cases

treatment of scca vulva25
Treatment of SCCA Vulva

Radiation Therapy:

Indicated if positive margins after WRE if re-excision not possible or desirable (i.e. around the clitoris or anal sphincter)

Indicated if positive inguinal/pelvic nodes

Radiation in combination with chemotherapy is an alternative to surgery in women with stage III/IVA disease

treatment of scca vulva26
Treatment of SCCA Vulva

Chemotherapy:

Indicated for metastatic disease (stage IVB)

Similar regimens as those used for metastatic cervical cancer

Platinum-based

Treatment is palliative

treatment of recurrent disease
Treatment of Recurrent Disease

Local recurrence treated with surgical re-excision

For large local recurrence, pelvic exenteration may be required

Inguinal lymph node recurrences have a much worse prognosis - treated with surgery and/or radiation therapy

Chemotherapy used for distant disease

melanoma of the vulva
Melanoma of the Vulva

2nd most common type of vulvar cancer (5-6%)

Occurs more frequently in white women

Mean age at diagnosis is 68y (in contrast, cutaneous melanomas of other sites usually develop before age 45y)

Usually pigmented lesion but amelanotic lesions also occur

Treatment is wide local excision with 2 cm margins and sentinel lymph node biopsy +/- treatment with chemotherapy and/or biologic agents

basal cell carcinoma
Basal Cell Carcinoma

2% of vulvar cancers

Usually occur in white, postmenopausal women

May be locally invasive but usually do not metastasize

Slow-growing

Treatment is wide local excision

paget disease of the vulva
Paget Disease of the Vulva

<1% of vulvar malignancies

Most patients are postmenopausal and Caucasian

Similar in appearance to Paget disease of the breast

Most common presenting symptom is pruritus

Lesion is usually well demarcated with slightly raised edges and a red background, dotted with small pale islands

Usually multifocal

paget disease of the vulva34
Paget Disease of the Vulva

Treatment is wide local excision

There is a high rate of local recurrence, even with negative surgical margins

Treatment for recurrence is re-excision

4-17% of women have an underlying invasive adenocarcinoma of the vulva

20-30% of women have a synchronous malignancy (breast, rectum, bladder, urethra, cervix or ovary)

summary vulvar cancer
Summary – Vulvar Cancer

Comprises 5% of gynecologic malignancies

2 pathways of vulvar carcinogenesis:

HPV infection (60%)

Chronic inflammatory (vulvar dystrophy)

Most common histology is squamous cell carcinoma

Treatment includes surgery, radiation and/or chemotherapy depending on stage