Flashcards in Vulval cancer Deck (29)
What proportion of gynaecological cancer does vulval cancer account for?
Most common type of vulval cancer?
Risk factors for vulval cancer
- HPV infection
- Lichen sclerosis
Lymphatic drainage of the vulva
- Primarily to inguinofemoral region
- Secondarily to external and internal iliac nodes
- Cancer can have unilateral or bilateral spread
Which other areas also share lymphatic drainage with the vulva?
inferior third of the vaginal tube and the most external portion of the anus (below the anal sphincter)
Primary prevention of vulval cancer
HPV vaccination (HPV 16 most common subtype causing HSIL and SCC)
Secondary prevention of vulval cancer
- No screening
- Self examination if they have lichen sclerosis
- women who are known to have squamous intraepithelial lesion (SIL) of the cervix, vagina, or anus should have inspection of the vulva as part of their follow‐up colposcopy visits
- Women with signs or symptoms of vulval cancer should have early biopsy
Two main pathological pathways that lead to vulvar SCC
1. Keratinizing SCC usually occurs in older women and is often associated with lichen sclerosus and/or differentiated vulvar intraepithelial neoplasia (dVIN).
2. Warty/basaloid SCC
- occurs in younger women,
- persistent infection with oncogenic strains of HPV (particularly HPV 16, 18, 31 and 33),
- has SIL as its precursor lesion.
- Lesions are frequently multifocal,
- HIV infection and cigarette smoking are also common predisposing factors
3 subtypes of premalignant lesions of the vulva
- low‐grade squamous intraepithelial lesions (LSIL);
- high‐grade squamous intraepithelial lesions (HSIL);
- differentiated VIN
Treatment of dVIN
- More likely to become malignancy than HSIL/LSIL
- Less likely to be associated with HPV
- Faster progression
Therefore- recommend surgical excision with 5‐mm margins and 4‐mm depth
Non surgical options: (reduce psychosexual morbidity)
Disadvantage- can't assess for occult invasion
1. Tumor confined to the vulva
IA Lesions ≤2 cm in size, confined to the vulva or perineum and with stromal invasion ≤1.0 mm, no nodal metastasis
IB Lesions >2 cm in size or with stromal invasion >1.0 mm, confined to the vulva or perineum, with negative nodes
FIGO stage 2
Tumor of any size with extension to adjacent perineal structures (lower third of urethra, lower third of vagina, anus) with negative nodes
FIGO stage 3
3: Tumor of any size with or without extension to adjacent perineal structures (lower third of urethra, lower third of vagina, anus) with positive inguinofemoral nodes
IIIA With 1 lymph node metastasis (≥5 mm), or
With 1–2 lymph node metastasis(es) (<5 mm)
IIIB With 2 or more lymph node metastases (≥5 mm), or
With 3 or more lymph node metastases (<5 mm)
IIIC With positive nodes with extracapsular spread
FIGO stage 4
4: Tumor invades other regional (upper 2/3 urethra, upper 2/3 vagina), or distant structures
IVA Tumor invades any of the following:
upper urethral and/or vaginal mucosa, bladder mucosa, rectal mucosa, or fixed to pelvic bone, or
fixed or ulcerated inguinofemoral lymph nodes
IVB Any distant metastasis including pelvic lymph nodes
6 histopathological types of vulvar cancer
Squamous cell carcinoma (80%)
Basal cell carcinoma
Paget's disease of the vulva
Adenocarcinoma, not otherwise specified
Bartholin gland carcinoma
Grade of vulvar cancer
GX: Grade cannot be assessed
G1: Well differentiated
G2: Moderately differentiated
G3: Poorly or undifferentiated
Principles of treatment
- Depends primarily on histology and staging.
- Other variables: age, coexistence of comorbidities, and performance status of the patient.
Treatment is predominantly surgical, particularly for SCC.
Concurrent chemoradiation is an effective alternative, for advanced tumors, and those where exenteration would be necessary to achieve adequate surgical margins.
Management should be individualized, and carried out by a MDT in a cancer center
Other therapies such as chemotherapy and immunotherapies are usually reserved for metastatic or palliative settings, or for the treatment of rare histologies such as melanoma
Symptoms of vulvar cancer
- May be asymptomatic,
- vulvar pruritus or pain,
-Lump or ulcer
-abnormal bleeding or discharge
-May have underlying lichen sclerosus or HSIL.
- Advanced vulvar cancer may present with a lump in the groin due to lymph node metastases.
Vulvar biopsy- try not to excise whole lesion if small as make subsequent surgery harder
Who is suitable for WLE?
- Lesion <2cm
- Depth of stromal invasion < 1mm
Groin node dissection not necessary
WLE as effective as vulvectomy but has less psychosocial morbidity
Aim for surgical margins of 2cm (pathological of 8mm)
1. Cervical cytology, and colposcopy of the cervix and vagina, due to the association of HPV‐related cancers with other squamous intraepithelial lesions.
2. Full blood count, biochemical profile, liver profile, and HIV testing.
3. Chest X‐ray.
4. CT or MRI scan of the pelvis and groin
5. 18F‐FDG PET‐CT can more effectively assess and detect inguinofemoral lymph node involvement compared with CT, influencing the planning of primary surgery and inguinal lymph node dissection to determine the optimum surgical extent without sentinel lymph node dissection and use of frozen sections. Additionally, PET‐CT might be used with larger tumors when metastatic disease is suspected or in the recurrence scenario.
Management: stage 1b or 2
All women who have Stage IB or Stage II cancers should have an inguinofemoral lymphadenectomy.
Management of small lateral lesions (less than 4 cm and ≥2 cm from the vulvar midline)
Ipsilateral groin dissection as <1% will have contralateral groin nodes
- tumor closer to (<2 cm) or crossing the midline,
- very large lateral tumors (>4 cm),
- positive ipsilateral nodes
bilateral groin node dissection
Indications for a sentinel node procedure, as per the GROINSS‐V study
Unifocal tumors confined to the vulva
Tumors less than 4 cm in diameter
Stromal invasion more than 1 mm
Clinically negative groin nodes
How are sentinel nodes detected?
Sentinel lymph nodes are identified using both radio‐labelled technetium and blue dye
indications for pelvic and groin irradiation in patients with positive groin nodes are:
Presence of extracapsular spread.
Two or more positive groin nodes
Target inguinofemoral and external and internal iliac lymph nodes with ERBT. Brachytherapy can also be used some times.
Management of advanced vulvar cancer
- Identify nodal involvement pre-op with FNA and PET-CT/MRI or CT
- No nodes identified- perform blateral lymphadenectomy. If no nodes positive then radiotherapy to nodes not necessary.
- If patient has multiple comorbidities/surgery inappropriate then primary chemoradiation may be used to treat the primary tumor as well as the groin and pelvic nodes
- surgical excision of the primary tumor with clear surgical margins and without sphincter damage, whenever possible.