21. Vulval Cancer

Question 1

How common is vulval cancer?

Answer 1

It accounts for 3-5% of all gynecological malignancies, with an incidence of 0.5-2 per 100,000 annually.

Question 2

What is the typical age group affected by vulval carcinoma?

Answer 2

Historically postmenopausal women (peak at 75 years), but now seen in younger women due to HPV-related VIN/HSIL, especially in the setting of HIV.

Question 3

What is the most common type of vulval cancer?

Answer 3

Squamous cell carcinoma (90% of cases).

Question 4

What is the second most common vulval cancer type?

Answer 4

Malignant melanoma.

Question 5

Name some rare histological types of vulval cancer.

Answer 5

Verrucous carcinoma
Paget’s disease of the vulva
Adenocarcinoma (NOS)
Bartholin’s gland carcinoma
Basal cell carcinoma
Sarcomas

Question 6

What are the common presenting symptoms of vulval carcinoma?

Answer 6

• Lump or ulcer (may be painful)
• Vulval pruritus (associated with VHSIL or lichen sclerosus)
• Bleeding or discharge
• May be asymptomatic in early stages

Question 7

What are the routes of spread in vulval carcinoma

Answer 7

1. Direct extension to adjacent structures (vagina, urethra, anus)
2. Lymphatic spread to inguino-femoral, then pelvic lymph nodes
3. Haematogenous spread (late, distant metastases)

Question 8

How is vulval carcinoma diagnosed?

Answer 8

Histology from a Keyes biopsy (local anaesthesia) or incisional biopsy.

Question 9

What are other investigations to consider?

Answer 9

• Pap smear (cervical screening)
• Blood tests: FBC, creatinine, ALP, GGT, HIV, VDRL/TPHA
• Chest X-ray
• Ultrasound/CT scan (abdomen, pelvis, groins) to assess spread

Question 10

What are the two distinct pathways to vulval squamous cell carcinoma?

Answer 10

In younger patients: Associated with HPV infection, HPV-related VIN (vulval HSIL), and cigarette smoking; tends to be multifocal.

In older patients: Often not associated with HPV, arises from differentiated VIN (not HPV-related), typically in areas affected by Lichen Sclerosus (LS), and is more likely to be unifocal.

Question 11

How is vulval squamous cell carcinoma staged?

Answer 11

Staging is according to the International Federation of Gynaecology and Obstetrics (FIGO) system.

Surgical staging is used, as clinical examination may miss involvement of groin lymph nodes.

Question 12

What is the staging for Stage 1 Vulval Squamous Cell Carcinoma?

Answer 12

Stage 1: Tumour confined to the vulva

1A: Lesions ≤ 2 cm, confined to the vulva or perineum with stromal invasion ≤ 1.0 mm, no nodal metastases.

1B: Lesions ≥ 2 cm or with stromal invasion > 1.0 mm, confined to the vulva or perineum, with negative nodes

Question 13

What is the staging for Stage 2 Vulval Squamous Cell Carcinoma?

Answer 13

Stage 2: Tumour of any size with extension to adjacent perineal structures (lower 1/3 urethra, lower 1/3 vagina, anus) with negative nodes.

Question 14

What is the staging for Stage 3 Vulval Squamous Cell Carcinoma?

Answer 14

Stage 3: Tumour of any size with or without extension to adjacent perineal structures with positive inguino-femoral lymph nodes.

3A: (i) With 1 lymph node metastasis (≥5mm), or (ii) 1-2 lymph node metastasis(es) (≥5mm).

3B: (i) With 2 or more lymph node metastases (≥5mm), or (ii) 3 or more lymph node metastases (<5mm).

3C: With positive nodes with extra-capsular spread.

Question 15

What is the staging for Stage 4 Vulval Squamous Cell Carcinoma?

Answer 15

Stage 4: Tumour invades other regional or distant structures.

4A: Tumour invades any of the following:
- Upper urethral and/or vaginal mucosa, bladder mucosa, rectal mucosa, or fixed to pelvic bone, or
- Fixed or ulcerated inguino-femoral lymph nodes.

4B: Any distant metastases, including pelvic lymph nodes.

Question 16

What is the cornerstone of treatment for Vulval Squamous Cell Carcinoma (SCC)?

Answer 16

The cornerstone of treatment is surgery, which includes:

1. Radical wide local excision of the primary tumour or radical vulvectomy.
2. Uni- or bilateral inguino-femoral lymph node dissection

Question 17

How is the treatment approach adjusted for smaller, unifocal tumours?

Answer 17

For unifocal tumours less than 4cm in size, the sentinel node technique may be used to minimise the morbidity of a full groin node dissection. Only an ipsilateral groin node dissection is required for lateral tumours. For central lesions, a bilateral groin node dissection is always indicated.

Question 18

What is the approach when groin lymph nodes are positive?

Answer 18

If the groin lymph nodes are positive, the patient will be offered radiotherapy to the groin nodes and the pelvis.

Question 19

What is the treatment for large or inoperable lesions?

Answer 19

For large or inoperable lesions, or those requiring exenterative surgery to obtain adequate excision of the primary tumour, concurrent chemoradiation (modified Nigro regime) can be given pre-operatively to shrink the tumour. Less radical surgery may follow if appropriate.

Question 20

The prognosis correlates with FIGO stage, and lymph node status is the most important prognostic factor.

Answer 20

Negative lymph nodes: >90% 5-year survival rate.

Positive lymph nodes: ~50% 5-year survival rate.

Question 21

What are the common sites for recurrence of vulval SCC?

Answer 21

Recurrence may occur:

1. Locally, in the primary site.
2. In the groin nodes.
3. Rarely, in distant sites.

Question 22

What are the treatment options for recurrent vulval SCC?

Answer 22

Local recurrences: Can be treated with excision or irradiation.

Groin recurrences: Usually treated with radiotherapy, if the patient has not received radiotherapy before

Question 23

What is the second most common neoplasm of the vulva?

Answer 23

Malignant melanoma is the second most common neoplasm of the vulva. It is staged using the Clarke and Breslow staging systems, as for melanomas elsewhere on the body.

Question 24

How is malignant melanoma of the vulva treated?

Answer 24

If possible, radical wide local excision with margins of at least 1cm around the lesion should be performed. The role of lymph node dissection in vulval melanomas is controversial.

Question 25

What should be done with all new pigmented vulval lesions?

Answer 25

All new pigmented vulval lesions should be excised and sent for histological evaluation.

Question 26

What types of cancers can arise from the Bartholin’s gland?

Answer 26

Bartholin’s gland cancers can arise from:

1. The duct, as transitional or squamous carcinomas.
2. The gland, as adenocarcinomas.
3. Adenoid cystic and adenosquamous types have also been reported.

Question 27

How is Bartholin’s gland cancer diagnosed?

Answer 27

The diagnosis is often made after resection of what was initially thought to be a persistent or recurrent Bartholin’s gland cyst.

Question 28

How is Bartholin’s gland cancer treated?

Answer 28

Treatment is similar to SCC of the vulva. However, due to the anatomical location of these tumours, obtaining adequate surgical margins is more challenging.

Question 29

What is Paget’s disease of the vulva?

Answer 29

Paget’s disease is a rare condition, mainly occurring in postmenopausal women. It is typically intraepithelial but may be associated with an underlying adenocarcinoma in the surrounding skin or in a pelvic or abdominal organ (e.g., colon, uterus, ovary).

Question 30

What symptoms do women with Paget’s disease present with?

Answer 30

Women usually present with vulval pain and pruritus. On examination, an erythematous, weeping, eczematoid lesion is often seen.

Question 31

How is Paget’s disease diagnosed?

Answer 31

Diagnosis is confirmed through biopsy. Further investigations such as pelvic ultrasound or colonoscopy are required to exclude underlying cancers.

Question 32

What is the treatment for Paget’s disease?

Answer 32

The mainstay of treatment is radical excision. This can be challenging, as the disease often extends beyond the macroscopic lesion. In some cases, Imiquimod can be used to treat intraepithelial disease.