Vulval Cancer and Lichen Sclerosis

Question 1

How common is vulval cancer and who most commonly gets it?

Answer 1

Uncommon (approximately 3% of female genital cancers). Approximately 2/3rds occur in women over 60 years of age.

Question 2

What are the histological types of vulval cancer?

Answer 2

Types: squamous cell carcinoma – most common type, extramammary Paget’s disease, basal cell carcinoma and malignant melanoma.

Question 3

What is the most common cause of vulval squamous cell cancer?

Answer 3

Approximately 30% are related to HPV infection, usually HPV 16. Peak age: 6th decade. Risk factors the same as for cervical carcinoma. Approximately 70% are unrelated to HPV infection – Peak age: 8th decade. Often occur in longstanding inflammatory and hyperplastic conditions of the vulva, e.g. lichen sclerosis.

Question 4

Where do vulval cancer usually spread to?

Answer 4

Spreads initially to inguinal, pelvic, iliac and para-aortic lymph nodes. Also, to lungs and liver. Lesions less than 2cm - 90% five-year survival following vulvectomy and lymphadenectomy.

Question 5

What is VIN and how does it present?

Answer 5

Dysplastic lesions of the squamous epithelium in the vulva. Usually presents with pain or itching but can be asymptomatic. May be raised and warty or flat and erythematous.

Question 6

How is VIN diagnosed?

Answer 6

Diagnosis made by punch biopsy or excision biopsy.

Question 7

How is VIN treated?

Answer 7

Surveillance – biopsy of anything suspicious
Surgery – excision
Immunotherapy cream has been shown to help and reduced the need for excision

Question 8

What are the risk factors for vulval cancer?

Answer 8

• HPV
• VIN
• Herpes simplex virus type 2
• Lichen Sclerosis
• Smoking
• Immunosuppression
• Chronic vulvar irritation

Question 9

How does Vulval cancer usually present?

Answer 9

Presentation
Lump
Itchy 
Painful sores 
Ulceration 
Burning sensation
Bleeding  

Investigations
On examination LN may be palpable

Question 10

How should vulval cancer be managed?

Answer 10

Surgical excision and repair

Radiotherapy/chemotherapy – before to reduce size and after if positive LN found

Question 11

What is extra mammary paget’s disease?

Answer 11

Pruritic, red, crusted area usually on labia majora. Malignant cells, singly or in small clusters, along basal layer of epithelium. Usually no underlying cancer (unlike mammary Paget’s disease). This requires wide local excision.

Question 12

What is Lichen sclerosis?

Answer 12

Definition – chronic inflammatory skin disease most common in prepubescent girls and post-menopausal women. Can progress to squamous cell carcinoma

Question 13

What are the risk factors for lichen sclerosis?

Answer 13

Cause is unknown – maybe something to do with antibodies to extracellular matrix
• Genetics
• Other immune disorders

Question 14

How does lichen sclerosis present?

Answer 14

Asymptomatic
Itching leading to erosion
Dysuria
Dyspareunia if sexually active

Question 15

How does lichen sclerosis appear on examination?

Answer 15

Microscopy – atrophy producing thin epithelium
White atrophic patches most commonly on the skin around and on the vulva
Clitoral hood fusion
Fusion of the labia minor and majora
Posterior fusion resulting in loss of the vaginal opening
Figure of 8 appearance around vulva and anus

Question 16

How is lichen sclerosis diagnosed?

Answer 16

Diagnosis is made clinically

Biopsy can be performed if unclear

Question 17

How is lichen sclerosis managed?

Answer 17

Topical steroids – clobetasol propionate for 3 months
Immunosuppression if no improvement with Tacrolimus or other calcineurin inhibitor
Follow up due to increased cancer risk