Vulval carcinoma

Question 1

What is vulva intraepithelial neoplasia?

Answer 1

VIN is the pre-invasive phase of vulval carcinoma (with white areas with surrounding inflammation, may be itchy)

Question 2

How can VIN be subdivided?

Answer 2

Usual type VIN
-Most VIN
-Can be warty, basaloid or mixed
-Most common 35-55y
-Assoc with HPV (esp 16), CIN, smoking, chronic immunosuppression
-Clinically multifocal, red/white/pigmented, plaques/papules/patches, erosions/nodules, warty or hyperkeratosis
-Classically associated with warty or basaloid squamous cell carcinoma
Differentiated type VIN
-Rarer
-Assoc with lichen sclerosis, seen in older women
-Unifocal lesion as ulcer/plaque; linked to keratinizing squamous cell carcinoma of vulva
-Higher risk of progression to cancer vs usual type VIN

Question 3

What causes VIN?

Answer 3

Often HPV, typically 16
Warts may not be visible, but 5% acetic acid stains areas white
If VIN found on biopsy, examine cervix, anal canal if within 1.5cm, natal cleft skin and breasts (>10% have neoplasia elsewhere, most commonly cervical)

Question 4

How does VIN commonly present?

Answer 4

Pruritis or pain

Question 5

How is VIN treated?

Answer 5

Surveillance of suspicious lesions key
Painful lesions can be removed but vulvectomy/ablation not recommended due to high recurrence rate and poor functional outcome
Confirm histology and exclude invasive disease
Emollients or a mild topical steroid (e.g. imiquimod 5% cream) may help relieve symptoms (stimulates immune response); 2-3 times/week for 12w
Avoid vulval irritants
Therapeutic use of HPV vaccine, photodynamic therapy, IFN use and cavitron ultrasonic surgical aspiration techniques

Question 6

How common is vulval cancer?

Answer 6

5% genital tract pathologies
1200 new cases UK/yeasr
400 deaths

Question 7

What age group commonly get vulval cancer?

Answer 7

Over age 60, elderly class

Question 8

What type of tumours are vulval cancers?

Answer 8

90% squamous

Others are melanoma, basal cell carcinomas or carcinoma of Bartholin’s glands

Question 9

What can cause vulval carcinoma?

Answer 9

Most arise de novo
Lichen sclerosis
Immunosuppression
Smoking
Paget's disease of vulva

Question 10

How does vulval carcinoma present?

Answer 10

Lump
Indurated ulcer (may go unnoticed unless painful/bleeding - so many present late)
May be pre-invasive phase (VIN)
Exam classical demonstrates an ulcer/mass, typically on labia majora or clitoris
Hard, immobile inguinal LNs may be palpated

Question 11

How are vulval cancers staged?

Answer 11

Stage 1a - Confined to vulva/perineum (<2cm with stromal invasion; <1mm with negative LNs)
Stage 1b - Confined to vulva/perineum (>2cm or with stromal invasion >1mm; negative nodes)
Stage 2 - Any size with adjacent spread (lower urethra/vagina or anus); negative nodes
Stage 3 - Any size with positive inguinofemoral nodes
Stage 4 - Invades upper urethra/vagina, rectum, bladder, bone (4a) or distant mets (4b)

Question 12

How is vulval carcinoma treated?

Answer 12

Stage 1a - wide local excision without inguinal lymphadenectomy (spread risk negligible)
All other stages - sentinel lymph node biopsy or wide local excision and groin lymphadenectomy (triple incision radical vulvectomy)
Radiotherapy may be used to shrink tumours prior to surgery, especially if sphincters at risk of damage
Skin grafts (and liaison with plastics) may be needed

Question 13

What is the prognosis for vulval cancer?

Answer 13

5yr survival:

• At stage 1 = >90%
• At stages 3-4 = 40%

Question 14

What surgical options are available for vulval cancer?

Answer 14

Radical wide excision
Radical hemi/total vulvectomy
Radical anovulvectomy/posterior exenteration
Unilateral/bilateral inguinofemoral node dissection
Sentinel nodes
(Radio/chemo)