Vulval Cancer Flashcards
What are the two main types of vulval intraepithelial neoplasia (VIN)?
- Usual type VIN
- Differentiated type VIN
Regarding usual type VIN:
- What causes it?
- Who does it usually affect?
- Describe the different types
Caused by HPV types 16, 18 and 33.
Mainly occurs in women age 30-40 years and smokers.
LSIL: benign and self-limiting manifestations of HPV. Include flat condyloma.
HSIL: often multifocal, affecting interlabial grooves, posterior fourchette and perineum.
- Basaloid subtype: atypical immature parabasal type cells with numerous mitotic figures and enlarged hyperchromatic nuclei.
- Warty (condylomatous) type: undulating, spiking surface. Histology: cellular proliferation with numerous mitotic figures and abnormal maturation.
What % of usual type HSIL VIN will also have invasive SCC at the time of treatment?
10-22%
What are the implications of the embryonic origins of anogenital epithelium and the presence of VIN of the vulva?
It is derived from embryonal cloaca (cervix, vagina, vulva, anus, lower 3 cm of rectal mucosa up to dentate line).
- Women with VIN may have synchronus or metachronous squamous neoplasia of other lower genital tract sites e.g. cervix, vagina and anus.
Regarding differentiated type VIN:
- What causes it?
- Who does it usually affect?
- Describe its distribution and appearance
- Describe its histology
It arises from lichen sclerosus (<2% related to HPV infection). More likely to progress to cancer than usual type VIN.
Women age 50-60.
Often unifocal and unicentric.
Thickened epithelium and parakeratotic with elongated and anastomosing rete ridges.
Abnormal cell confined to parabasal and basal portion of rete pegs with little or no atypia above these layers.
Basal cells positive stain for p53.
What is the most common type of vulvar cancer?
Squamous cell carcinoma (90%)
What % of women with vulvar cancer are over 65 years old?
80%
What are the 2 main pathological pathways that lead to vulvar SCC?
- Keratinising SCC: from dVIN and lichen sclerosus. Occurs in older women.
- Warty/basaloid SCC: from persistent oncogenic HPV infection (types 16, 18 , 31, 33). OCCURS IN YOUNGER WOMEN.
What skin conditions are precursors to vulvar SCC?
- Lichen sclerosis
- oncogenic HPV
- Paget’s disease (preinvasive lesions of adenocarcinoma of the vulva), lichen planus (rare)
What are the risk factors for progression of VIN to cancer?
- Age >40
- Smoking
- previous CIN or cervical cancer
- Pelvic irradiation
- Immunosuppression
- Proximity to anal margin and SCJ
What is the second most common histopathological type of vulvar cancer?
Melanoma (5%)
What is the lymphatic drainage of the vulva?
Primary: inguinal and femoral lymph nodes
Secondary: internal and external iliac lymph nodes
Define stage I:
Define stage IA and IB:
- Stage I: confined to vulva
- Stage IA: <=2 cm in size, stromal invasion <1 mm
- Stage IB: >2cm in size, stromal invasion >1 mm.
Define stage II:
Stage II: tumour extension to adjacent perineal structures (lower third of urethra, lower third of vagina, anus) but negative nodes.
Define stage III
Define stage IIIC
- Stage III: positive inguinofemoral nodes. With or without extension to adjacent perineal structures.
- Stage IIIC: positive nodes with extracapsular spread.
Define stage IV
Define stage IVA and IVB
- Stage IV: invasion of other regional (upper 2/3 urethra, upper 2/3 vagina) or distant structures
- Stage IVA: invasions of upper urethra, vagina, bladder, rectum, pelvic bone OR fixed or ulcerated inguinofemoral lymph nodes.
- Stage IVB: distant metastasis including pelvic lymph nodes.
What is a primary prevention strategy for vulvar cancer?
HPV vaccination
What are 3 secondary prevention strategies for vulvar cancer?
○ Women with lichen sclerosus should be encouraged to regularly self-examine.
○ Early evaluation of patients with signs (pigmentated lesions, irregular ulcers) or symptoms (chronic vulvar pruritis) of vulvar disease and skin biopsy.
○ Women known to have squamous intraepithelial lesions (SIL) of the cervix, vagina or anus should have inspection of vulva as part of colposcopy.
What are 3 tertiary prevention strategies for vulvar cancer?
- Management of lichen sclerosus
- dVIN: excision with 0.5-1 cm margins
- HSIL (usual type VIN): excision, laser vaporisation or imiquimod.
What is the recurrence rate, benefits and risks of simple excision of vulvar HSIL?
- Recurrence rate 20%
- Benefits: provides histology to exclude microinvasion.
- Risks: disfiguring with psychosexual morbidity.
What is the recurrence rate, benefits and risks of CO2 laser vaporaisation of vulvar HSIL?
- Recurrence rate 20-40%
Benefits:
- Preservation of anatomy: young, multifocal, involving clitoris, urethra, anus and introitus
Risks:
- No histology
- May not be available
- Destroys hair follicles
What is the recurrence rate, benefits and risks of imiquimod 5% treatment of vulvar HSIL?
- Recurrence rate 16%
Benefits:
- Avoids scarring
- Avoid sexual dysfunction
- Good for small lesions and recurrent lesions (avoids multiple excisions)
Risks:
- Ineffective if immunocompromised.
- Long course 16 weeks; adherence issues.
Side-effects: inflammation, erosions.
What are the clinical features of vulvar SCC?
- Vulval pruritis
- Vulval ulcer or mass
- Abnormal vulval bleeding or
- Groin lymphadenopathy (advanced disease)
What examination and investigations would you perform in a woman you suspect has vulvar cancer?
Exam:
- Colposcopy: vulva, cervix and vagina + cytology biopsies to exclude co-existing SILs.
- Palpate groin nodes
Investigations:
- Punch or wedge biopsy (do not excise whole lesion)
- Cervical cytology
- Bloods: FBC, U&Es, LFTs, HIV serology
- CXR: metastases.
- MRI or CT pelvis: nodes
- PET-CT: better than CT in detecting inguinofemoral lymph node disease. Replaces need for SLNM.
