Vulvar Cancer

Charles H. Matthews and Daniel G. Petereit

 Background

Approximately how many pts are affected by vulvar cancer per yr in the U.S.? What is the incidence of vulvar cancer in the U.S.?

~3,500 pts are affected by vulvar cancer per yr in the U.S. The incidence is 1/100,000 people.

Vulvar cancer accounts for what % of gyn malignancies? What % of all malignancies in women are vulvar malignancies?

Vulvar cancer represents 3%–5% of all gyn malignancies. This comprises 1%–2% of all cancers in women.

What are the risk factors for vulvar cancer?

Risk factors for vulvar cancer:

1. 
   

   Increasing age

   
   
2. 
   

   HPV

   
   
3. 
   

   Vulvar intraepithelial neoplasia (VIN)

   
   
4. 
   

   Bowen Dz (squamous cell CIS)

   
   
5. 
   

   Paget Dz (lesions arising from Bartholin, urethra, or rectum)

   
   
6. 
   

   Erythroplasia

   
   
7. 
   

   Chronic vaginitis

   
   
8. 
   

   Leukoplakia

   
   
9. 
   

   Smoking

   
   
10. 
    

    Employment in laundry facilities

    
    
11. 
    

    Immune deficiency

What HPV subtypes are associated with vulvar cancer?

HPV subtypes associated with vulvar cancer include 6, 16, 18, and 33.

What is the function of HPV-associated oncoproteins?

It is thought that HPV-associated oncoproteins bind and inactivate tumor suppressor proteins such as Rb, p53, and p21.

What are the 7 subsites of the vulva?

Subsites of the vulva:

1. 
   

   Labia majora

   
   
2. 
   

   Labia minora

   
   
3. 
   

   Mons pubis

   
   
4. 
   

   Clitoris

   
   
5. 
   

   Vaginal vestibule

   
   
6. 
   

   Perineal body

   
   
7. 
   

   Posterior forchette

What are the most common presenting Sx of pts with vulvar cancer?

Common presenting Sx of vulvar cancer: pruritis, vulvar discomfort or pain, dysuria, oozing or bleeding, and difficulty with defecation

In which subsites does vulvar cancer most commonly arise?

70% of vulvar cancers arise from the labia majora/minora.

How is “locally advanced” vulvar cancer defined?

Locally advanced vulvar cancer is defined as a vulvar tumor burden that cannot be resected without exenterative surgery.

What % of vulvar cancers are locally advanced at Dx?

30% of vulvar cancers are locally advanced at Dx.

What are the 1^st-, 2^nd-, and 3^rd-echelon LN regions in vulvar cancer, and which subsite is associated with skip nodal mets?

LN regions in vulvar cancer:

1. 
   

   1^st echelon: superficial inguinofemoral

   
   
2. 
   

   2^nd echelon: deep inguinofemoral and femoral

   
   
3. 
   

   3^rd echelon: external iliac nodes

The clitoris can drain directly to the deep inguinofemoral or pelvic nodes.

What are the 2 strongest predictors of LN involvement in vulvar cancer?

The 2 strongest predictors of LN involvement in vulvar cancer are tumor grade and DOI.

Estimate the risk of inguinal LN involvement based on the DOI of a cervical tumor: <1 mm, 1–3 mm, 3–5 mm, and >5 mm.

LN involvement by cervical tumor DOI:

1. 
   

   <1 mm: <5%

   
   
2. 
   

   1–3 mm: 8%

   
   
3. 
   

   3–5 mm: 27%

   
   
4. 
   

   >5 mm: 34%

(Hacker NF et al., Cancer 1993)

Estimate the risk of inguinal LN involvement based on the vulvar cancer FIGO stage.

LN involvement by the vulvar cancer FIGO stage:

1. 
   

   Stage I: 17%

   
   
2. 
   

   Stage II: 40%

   
   
3. 
   

   Stage III: 30%–80%

   
   
4. 
   

   Stage IV: 80%–100%

What histology constitutes the vast majority of vulvar cancers? Name 3 other histologies of tumors found on the vulva.

The most common vulvar histology is squamous cell carcinoma (80%–90%). Other histologies include melanoma, basal cell, Merkel cell, sarcoma, and adenocarcinomas of the Bartholin glands.

What % of vulvar cancers are multifocal?

~5% of vulvar cancers are multifocal.

 Workup/Staging

What is the Bx approach for small (<1 cm) vulvar lesions?

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