Vulvar cancer accounts for about 4% of gynecologic cancers. The most common type is squamous cell carcinoma, which typically affects women ages 65-75. Risk factors depend on whether the cancer is related to HPV infection or vulvar dystrophy. Treatment involves surgical excision, with laser ablation and wide local excision being main options. Topical imiquimod and 5-fluorouracil can also be used to treat preinvasive high-grade lesions. Long-term surveillance after treatment is important due to the risk of recurrence.

1. Vulvar cancer
Tevfik Yoldemir MD BSc MA PhD
tevfikyoldemir
profdrdrtevfikyoldemir

2. Age at diagnosis

3. Estimated new gynecologic cancer cases and
deaths in the United States in 2008

4. Stage at presentation

5. Age at death

6. Average 5-year survival by stage

7. Epidemiology
• Vulvar carcinoma accounts for approximately 4 % of gynecologic malignancies.
• In the USA during 2013, there will be 4,700 new diagnoses of vulvar cancer and
approximately 990 related deaths [ 1 ].
• The most common histology is squamous cell carcinoma (SCC) followed by
melanoma and adenocarcinoma.
• Peak incidence is between 65 and 75 years of age and the median age at
diagnosis is 68.
• Recent series suggest vulvar cancers etiologically related to human
papillomavirus infection (HPV) infection present at a younger age than non-HPV
related cancers.
• Risk factors for invasive vulvar cancer depend on two distinct etiologic
pathways:
– Keratinizing, well-differentiated carcinomas arise in the background of vulvar
dystrophy, such as lichen sclerosus or squamous hyperplasia.
– Non-keratinizing carcinomas develop from malignant transformation of
dysplastic conditions related to HPV infection, smoking, or immunosuppresion.
– Quadrivalent HPV vaccination is effective against HPV types 16, 18, 6, and 11
and the expectation is that immunization will decrease the incidence of vulvar
cancers related to HPV in the future

8. Current terminology by the International
Society for the Study of Vulvar Diseases (2004)
• VIN, usual type (warty, basaloid, or mixed).
• VIN, differentiated is now used to describe what was
previously referred to as VIN simplex type (not HPV related).
• VIN, unclassified encompasses VIN that cannot be classified
into either of the above groups including pagetoid type cells.
• The VIN 1 category was eliminated (because the diagnosis is
not reliably reproducible and the findings are associated with
HPV effect or reactive changes, not a precancerous lesion).
• VIN 2 and VIN 3 were combined since they are difficult to
differentiate and would both be treated as high-grade
preinvasive dysplasia.

9. • Surgical excision is the mainstay of therapy for high-grade lesions of
the vulva. Options include wide local excision, simple vulvectomy,
and CO 2 laser ablation.
– Wide local excision is warranted if invasion cannot be excluded and is
the preferred treatment of differentiated VIN because of the high
malignant potential.
– Laser ablation is beneficial in extensive multifocal lesions or if it is
not feasible to obtain adequate margins with excision.
○ The CO 2 Laser using colposcopic guidance achieves precise control
over the depth of desired destruction of 1–2 and 2–3 mm, for non-
hair-bearing and hairbearing areas, respectively. The intraepithelial
lesion is effectively treated by vaporizing the epidermis, papillary
dermis, and superficial reticular dermis, but the minimal residual
thermal damage allows rapid healing with very little scarring.
Postoperative pain management: oral narcotic analgesics, sitz baths,
and topical 1 % lidocaine and 2 % silver sulfadiazine cream.

10. • Imiquimod
– Topical immune response modulator that affects local cytokine
production and cell-mediated immunity that have indirect antitumor
and antiviral effects.
– Dosing and administration: 0.25 g Imiquimod 5 % cream is applied
topically only to the lesion 2–3 times per week before bedtime for 16
weeks, but the schedule can be modified based on side-effects and
response. Sulfur precipitate 5 % in zinc oxide ointment can be applied
the day after imiquimod to avoid super- infection .
– Adverse reactions are related mostly to inflammation at application
site consisting of mild to moderate erythema, pruritis, erosion or
painful ulceration.
– Several randomized controlled trials have shown that imiquimod is
more effective than placebo for the treatment of VIN but it is still
considered investigational for this purpose.

11. • Topical 5-Fluorouracil (5-FU)
– 5-FU is a topical cream that causes chemical desquamation of VIN
lesion and is very effective with response rates as high as 75 % but is
not as well tolerated compared to other topical agents and is more
commonly used for vaginal dysplasia.
– Monitoring for intraepithelial or invasive squamous neoplasia every 6
months includes cervical and vaginal cytology (anal pap smears if
involved) and careful inspection of the lower genital tract with
colposcopy as needed. Smoking cessation and HIV testing should be
encouraged.
– Approximately one-third of women will have recurrent VIN following
treatment regardless of the modality.
– Long-term surveillance is recommended at 3 and 6 months following
treatment then every 6 months for a total of 5 years and then annually
thereafter.

12. VCa - Clinical presentation
○ Vulvar cancer may be asymptomatic but pruritis is the most
common symptom.
○ Approximately 50 % present with a lump or ulcer on the vulva
(or less commonly in the groin from metastases to lymph nodes).
○ Clinicians should have a low threshold to biopsy any suspicious
vulvar abnormalities, because the appearance of malignant
lesions is often similar to that of benign processes.

13. Pretreatment evaluation
• Pathologic diagnosis is obtained using wedge or Keyes biopsy.
• Clinical assessment with thorough history and physical exam
including palpation of groin lymph nodes and complete pelvic
with Pap smear if cervix remains in situ and colposcopy of the
entire cervix, vagina, and vulva.
• Imaging with PET or MRI for the evaluation of lymph nodes and
soft tissues as appropriate.
• Imaging is more sensitive than physical exam for detecting
inguinal lymph node involvement; however, inflammatory
processes may lead to false positive findings.
• Prior to initiating treatment, it is important to disclose the risks
and benefits of treatment with particular attention to
counseling on sexual function after treatment.

14. Staging

15. Staging

18. Relative risks of In Situ and Invasive Vulvar
Cancer