Vulvar cancer Flashcards
Incidence of vulvar cancer. Peak age of incidence
- Vulval cancer is rare, with just over 1000 cases newly diagnosed in the UK per year. It is ranked as the 20th most common female cancer.
- Affects predominantly elderly women and is uncommon below the age of 50 years. Comorbidities also increase with age
- By age group, the incidence trend has remained relatively stable over the last three decades, although the incidence in women aged 40–49 years has risen two-fold.
Most common type of vulvar cancer
- 90% of all vulval cancers are squamous cell carcinomas
- Melanoma, Padget’s disease, Bartholin gland tumours and basal cell carcinomas account for remaining 10%
How does vulvar cancer spread
- Vulvar cancer spreads by direct extension to adjacent structures, embolization to the inguinal and femoral nodes (regional lymph nodes) or by haematogenous spread- 30% of women with operable disease have nodal spread
What is VIN
VIN= Vulval intraepithelial neoplasia (presence of atypical cells in the vulval epithelium)
Pathophysiology of squamous cell carcinomas of the vulva
High-risk HPV associated (HPV16) (usual type VIN)
* Arise on a background of high-grade vulval intraepithelial neoplasia (VIN3)
* Usually young women (aged 35-55)
* Other RFs: smoking, immunosuppression
* Leads to warty or basaloid SCC
* Usually multifocal, with heterogenous appearance (red, white or pigmented plaques, papules, erosions, nodules)
Non-HPV associated (differentiated type VIN)
* Usually older women
* Associated with Lichen sclerosis (risk is approximately 4%)- not clear if risk is reduced by treatment
* Leads to keratinising SCC of vulve
* Usually unifocal, presenting as an ulcer or plaque
Presentation of vulval cancer
- Presentation will vary according to the stage of disease. Women often have difficulty articulating vulval symptoms and all women with vulval symptoms should be examined
- Diagnosis may be incidental during examination or catheterisation
- Symptoms include vulval itching, irritation or pain. Women may also notice a lump, bleeding or discharge.
- If an unexplained vulval lump is found, an urgent referral should be made (within the 2 week wait schedule).
- Vulval cancer may also present with vulval bleeding due to ulceration (should also be referred under -week-wait)
- Most commonly masses present on labia majora or clitoris
- Enlarged, immobile and hard inguinal lymph nodes
- Any change in the vulval epithelium in a postmenopausal woman warrants a biopsy
Differentials for vulval cancer
(ulceration of vulva)- Infection, Inherent skin conditions (Eczema, Lichen sclerosis, Intra-epithelial neoplasia)
Investigations for vulval cancer
- History and examination (size, position (unilateral, bilateral, midline), groin lymph nodes)
- Swabs for culture if suspected infection
- All diagnoses should be based upon a representative biopsy of the tumour- should include the area of epithelium where there is a transition of normal to abnormal tissue.
- Diagnostic biopsies should be >1mm in depth- differentiate superficial and invasive tumours
- In women where a vulval cancer is strongly suspected on examination, urgent referral to a cancer centre should not await biopsy
Staging of vulval cancer
Staged using the FIGO staging system:
Stage 1- Tumour confined to the vulva
* 1a- Lesions <2cm in size, confined to the vulva or perineum and with stromal invasions <1mm
* 1b- Lesions >2cm in size OR >11mm of stromal invasion
Stage 2- Tumour of any size with extension to adjacent perineal structures (lower 1/3 urethra, lower 1/3 vagina, anus) with negative nodes
Stage 3- Tumour of any size with or without extension into adjacent perineal structures, with positive inguinofemoral nodes
* 3a- with 1 lymph node metastasis (>5mm) or 2 (<5mm)
* 3b- With 2 or more lymph node metastases (>5mm) or 3 (<5mm)
* 3c- with positive nodes with extracapsular spread
Stage 4- Tumour invades other regional (upper 2/3 urethra, upper 2/3 vagina) or distant structure (4b)
Management of vulval cancer
- For early-stage disease: Stage 1a can be managed by wide local excision only, without groin or node dissection (risk of metastasis is negligible)
- Stage 1b or layer dissection of groin nodes should be performed
- Surgery to primary tumour should be radical enough to remove the tumour with adequate margins (At least a 15 mm margin)
- Excision of atypical skin (lichen sclerosus or VIN) affecting the remainder of the vulva should be considered
- For late-stage disease: Wide, radical, local excision with a minimum of 15 mm disease free margin may be used but some tumours will require a radical vulvectomy.
- If there is risk of sphincter damage and faecal incontinence, radiotherapy should be considered to shrink tumour volume.
What is meant by a sentinal lymph node biopsy, when is it used (what is the alternative)
- Untreated groin node metastases will be fatal and affected nodes cannot be reliably identified with radiology
- Current approach: full inguinofemoral lymphadenectomy (for all tumours with depth of invasion > 1 mm)
- NOTE: groin lymphadenectomy is a very morbid procedure with complications including wound healing problems, infection, VTE and chronic lymphoedema
- Groin nodes are involved in 15% of women with vulval cancer
- Full groin lymphadenectomy may be avoided by doing a sentinel lymph node biopsy (first node that the area drains to)
- A dye and radioactive nucleotide can be injected into the vulval tumour to identify the sentinel node
- If the sentinel node is positive for disease, then full groin lymphadenectomy is indicated
- Should only be done in primary SCVC, where cancer is <4cm, macroscopic and unifocal and there is no clinical or radiological evidence of lymph node mets
What is the most common type of vaginal cancer, what is the incidence of vaginal cancer
Vaginal cancer is very rare. Tumours generally arise from metastatic spread from the endometrium and cervix. Primary cancers of the vagina are usually squamous cell carcinomas (sometimes clear cell adenocarcinomas and malignant melanomas may occur
What is the peak age of onset of vaginal cancer
- Peak age of onset is 60-70 years old
When can vaginal cancer occur in children
- Clear cell carcinomas are most common in children and associated with use of diethylstilbesterol (used to prevent miscarriage in the late 1950s- early 1970s) in mothers
What are some risk factors for vaginal cancer
- 60% of primary tumours are HPV-associated
- Other RFs: Previous malignant/pre-malignant disease of the cervix, Vaginal intraepithelial neoplasia (VaIN), previous pelvic radiotherapy
