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Gynae-Oncology > Vulval Cancer > Flashcards

Vulval Cancer Flashcards

1
Q

What are the two main types of vulval intraepithelial neoplasia (VIN)?

A
  • Usual type VIN (LSIL/HSIL)

- Differentiated type VIN

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2
Q

Regarding usual type VIN:

  1. What causes it?
  2. Who does it usually affect?
  3. Describe the different types
A

Caused by HPV types 16, 18, 31 and 33.

Mainly occurs in women age 30-40 years and smokers, HIV infection.

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.
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3
Q

What % of usual type HSIL VIN will also have invasive SCC at the time of treatment?

A

10-22%

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4
Q

Regarding differentiated type VIN:

What are key differences from usual type VIN?
Who does it usually affect?
Describe its distribution and appearance.
Describe its histology.

A

Caused by inflammatory skin conditions, rather than HPV. <2% related to HPV infection.

More likely to progress to cancer than usual type VIN, and over shorter time frame.

Postmenopausal 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.

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5
Q

What is the most common type of vulvar cancer?

A

Squamous cell carcinoma (90%)

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6
Q

What % of women with vulvar cancer are over 65 years old?

A

80%

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7
Q

What are the 2 main pathological pathways that lead to vulvar SCC?

A
  • 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.
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8
Q

What skin conditions are precursors to vulvar SCC?

A
  • Lichen sclerosis
  • dVIN
  • usual type VIN (LSIL/HSIL)
  • Paget’s disease (preinvasive lesions of adenocarcinoma of the vulva)
  • Lichen planus (very rarely)
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9
Q

What is the second most common histopathological type of vulvar cancer?

A

Melanoma (5%)

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10
Q

What is the lymphatic drainage of the vulva?

A

1st - inguinal and femoral LNs
2nd - internal and external lilac nodes

If clitoral lesions - may drain directly to iliac nodes

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11
Q

Define stage I:

Define stage IA and IB:

A
  • Stage I: confined to vulva
  • Stage IA: <=2 cm in size, stromal invasion <1 mm
  • Stage IB: >2cm in size, stromal invasion >1 mm.
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12
Q

Define stage II:

A

Stage II: tumour extension to adjacent perineal structures (lower third of urethra, lower third of vagina, anus) but negative nodes.

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13
Q

Define stage III

Define stage IIIC

A
  • Stage III: positive inguinofemoral nodes. With or without extension to adjacent perineal structures.
  • Stage IIIC: positive nodes with extracapsular spread.
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14
Q

Define stage IV

Define stage IVA and IVB

A

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.
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15
Q

What is a primary prevention strategy for vulvar cancer?

A

HPV vaccination
Smoking cessation
Safe sex practices e.g. condom use

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16
Q

What are 3 secondary prevention strategies for vulvar cancer?

A

○ 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.

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17
Q

What are 3 tertiary prevention strategies for vulvar cancer?

A
  • Management of lichen sclerosus
  • dVIN: excision with 5mm margins
  • HSIL (usual type VIN 2/3): excision with 5mm margins, laser vaporisation or topical imiquimod.
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18
Q

What is the recurrence rate, benefits and risks of simple excision of vulvar HSIL?

A
  • Recurrence rate 20% - lower than for imiquimod or CO2 laser therapy
  • Needs 6 monthly follow-up for at least 3 years
  • Benefits: provides histology to exclude microinvasion, has lowest rates of recurrence for 3 treatment options.
  • Risks: disfiguring, nerve damage, psychosexual morbidity, affects function (urination/defecation)
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19
Q

What is the recurrence rate, benefits and risks of CO2 laser vaporaisation of vulvar HSIL?

A
  • Recurrence rate up to 40% - higher than for surgical excision procedures

Benefits:

  • Preservation of anatomy, function and reduction in psychosexual sequelae
  • Indicated when: young patient, multifocal, involving clitoris, urethra, or anus (pt must be low risk for possible invasive disease)

Risks:

  • No histology
  • May not be available
  • Destroys hair follicles
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20
Q

What are the benefits and risks of imiquimod 5% treatment of vulvar HSIL?

A

Benefits:

  • Avoids scarring
  • Avoid sexual dysfunction
  • Good for small lesions and recurrent lesions (avoids multiple excisions)
Risks:
- No tissue diagnosis
- Ineffective if immunocompromised.
- Long course 16 weeks; adherence issues. 
Side-effects: inflammation, erosions.
21
Q

What are the clinical features of vulvar SCC?

A
  • Vulval pruritis
  • Vulval ulcer or mass
  • Abnormal vulval bleeding or
  • Groin lymphadenopathy (advanced disease)
22
Q

What examination and investigations would you perform in a woman you suspect has vulvar cancer?

A

Exam:

  • Cervical cytology
  • Colposcopy: vulva, cervix and vagina + 3-4mm punch biopsy any suspicious lesions.
  • Palpate groin nodes

Investigations:

  • 3-4 mm punch or wedge biopsy (do not excise whole lesion)
  • FNA clinically positive LN
  • Cervical cytology
  • Bloods: FBC, U&Es, LFTs, HIV serology
  • CXR: metastases.
  • MRI or CT pelvis: nodes
  • PET-CT: used in suspected metastatic disease or recurrence, as better than CT in detecting inguinofemoral lymph node disease. Replaces need for SLNM.
23
Q

What is the surgical management of a stage IA microinvasive vulvar cancer?

A

Radical wide local excision with resection margins of 2 cm.

No need for groin lymph node dissection.

24
Q

What is the surgical management of a stage IB to II vulvar cancer?

A

Radical wide local excision with resection margins of 2 cm.
- (Deep margin: inferior fascia of the urogenital diaphragm +/- distal 1 cm of urethra)

Inguinofemoral lymphadenectomy
Consider ipsilateral node dissection if:
  - single lesion, 
  - <4cm, 
  - >2cm from midline,
  - clinically negative nodes

OR sentinel node biopsy (as per GROINS IV study) if:

  • single lesion,
  • <4cm,
  • > 1mm depth invasion,
  • clinically negative nodes

If any nodes positive, requires bilateral LN dissection.

25
Q

Regarding patients with stage IB to II vulvar cancer:

  • Who is considered low risk and thereby ipsilateral groin dissection may be adequate?
  • Who is considered high risk thereby bilateral groin dissection is needed?
A

Low risk:

  • Small lateral lesions (single lesions, <4 cm size and >=2 cm from midline)
  • Justification: <1% of low risk patients with negative ipsilateral nodes will have metastases to the contralateral groin nodes.

High risk:

  • Lesions <2 cm from OR crossing midline
  • Involves anterior labia minora/clitorus
  • Very large tumours (>4 cm)
  • Clinically positive groin nodes.
26
Q

Regarding patients with stage IB to II vulvar cancer:

  • Outline the goals of sentinel node procedure
  • Outline patients appropriate for sentinel node procedure.
  • What is the recurrence % and disease specific survival after 3 years?
A

Goals:
- to detect nodal metastases in sentinel node that primarily drains the tumour and omit full lymphadenectomy in sentinel node negative patients.

Indications (GROINSS V study):

  • Unifocal tumour confined to vulva
  • < 4cm in diameter
  • Stromal invasion >1 mm
  • Clinically negative groin nodes

Note: if unable to detect ipsilateral sentinel node complete ipsilateral inguinofemoral lymphadenectomy is required.
If sentinel node is positive = complete BILATERAL inguinofemoral lymphadenectomy is required.

Recurrence rate 2.3%
Disease specific survival after 3 years: 97%

27
Q

After resection of tumour, if histology shows close margins (<5 mm), how should this patient be managed?

A

With adjuvant radiotherapy as there is a high rate of recurrence associated with close margins.

28
Q

Which type out of usual type VIN and differentiated type VIN is associated with a higher risk of malignant transformation?

A

Differentiated

Approx 60%

29
Q

What proportion of gynaecological cancer does vulval cancer account for?

A

2-5%

30
Q

Risk factors for vulval cancer

A
  • Postmenopausal
  • Oncogenic HPV infection
  • smoking
  • HIV
  • Lichen sclerosis
  • dVIN
  • HSIL/LSIL
  • Immune suppression
  • lichen planus
31
Q

Which other areas also share lymphatic drainage with the vulva?

A

inferior third of the vaginal tube and the most external portion of the anus (below the anal sphincter)

32
Q

Primary prevention of vulval cancer

A

HPV vaccination (HPV 16 most common subtype causing HSIL and SCC)

33
Q

Two main pathological pathways that lead to vulvar SCC

A
  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
34
Q

3 subtypes of premalignant lesions of the vulva

A
  • low‐grade squamous intraepithelial lesions (LSIL);
  • high‐grade squamous intraepithelial lesions (HSIL);
  • differentiated VIN
35
Q

Treatment of dVIN

A

surgical excision with 5‐mm margins and 4‐mm depth

36
Q

6 histopathological types of vulvar cancer

A 
Squamous cell carcinoma (80%)
Melanoma
Basal cell carcinoma
Verrucous carcinoma
Paget's disease of the vulva
Adenocarcinoma, not otherwise specified
Bartholin gland carcinoma
37
Q

Grade of vulvar cancer

A

GX: Grade cannot be assessed
G1: Well differentiated
G2: Moderately differentiated
G3: Poorly or undifferentiated

38
Q

Principles of treatment

A
  • 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

39
Q

Indications for a sentinel node procedure, as per the GROINSS‐V study

A

Unifocal tumors confined to the vulva
Tumors less than 4 cm in diameter
Stromal invasion more than 1 mm
Clinically negative groin nodes

40
Q

How are sentinel nodes detected?

A

Sentinel lymph nodes are identified using both radio‐labelled technetium and blue dye

41
Q

Indications for pelvic and groin irradiation:

A

Presence of LN extracapsular spread.
Two or more positive groin nodes.
Surgical margins ≤5mm.

Target inguinofemoral and external and internal iliac lymph nodes with ERBT. Brachytherapy can also be used some times.

42
Q

Management of advanced vulvar cancer

A
  • Identify nodal involvement pre-op with FNA and PET-CT/MRI or CT

If patient has multiple comorbidities or surgery likely to cause high morbidity (e.g. exenteration) 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.
  • bilateral inguinofemoral lymphadenectomy - removal of any enlarged nodes.
  • Stage IV disease - Pelvic exenteration surgery OR primary chemoradiotherapy with secondary surgery to remove residual disease.

Radiotherapy?
- All node positive disease should have adjuvant radiotherapy

Chemotherapy?
- Chemotherapy may improve outcomes in node positive disease too

43
Q

Indication for chemoradiation

A
  • If adequate excision of the primary tumor can only be achieved by exenteration and the formation of a bowel or urinary stoma, radiotherapy (with or without concurrent chemotherapy) may be a preferred treatment alternative.

Survival is improved if any postradiation residual tumor is resected

  • 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
44
Q

What chemotherapy agents have been used in vulval cancer?

A
  • Chemotherapy agents: cisplatin +/- 5-fluorouracil
45
Q

What is the management of Pagets disease of the vulva?

A

WLE (first-line).
Check for underlying primary genital tract/anal/urothelial carcinoma.

Conservative option = CO2 laser or imiquimod.

Recurrence rates are HIGH - thus long term follow-up recommended.

46
Q

What is the management of bartholins gland carcinoma?

A

Radical hemi-vulvectomy and bilateral groin node dissection.

+/- adjuvant radiotherapy

47
Q

What is the management of melanoma of the vulva.

A

WLE.

No evidence for LN dissection.

48
Q

What is the prognosis for recurrence after vulval cancer?

A
  • Up to 1/3 vulval cancers get recurrence
  • Primary site recurrence increased if surgical margins <8cm - typically within first 2 years
  • Remote site recurrences occur later > 5 years

Believed many recurrences are actually new cancers developing within a ‘field of cancerisation’ where premalignant cellular changes predispose to new malignant transformation.

49
Q

What is the follow-up for vulval cancers?

A

6 wk after surgery
3 monthly for year 1
6 monthly for year 2
Annually for at least 5 years - longterm follow-up not unusual

Gynae-Oncology (14 decks)

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