Malignant disease of the vulva

Question 1

1 - Lichen sclerosis has a malignancy risk of 30%

2 - A vulval biopsy should be considered when a woman fails to respond to topical treatment

3 - Most women with high-grade vulval intraepithelial neoplasia will develop vulval cancer if left untreated

4 - Vulval cancer is the second most common cancer of the female genital tract

5 - A quarter of vulval cancers present in women under 40 years.

Answer 1

1 - The answer is false. It is nearer 5%.

2 - The answer is true. Most benign conditions of the vulva will respond to topical treatment. Those that do not include VIN and malignancy and must be excluded as a diagnosis.

3 - The answer is false. 5% of treated high-grade vulval intraepithelial neoplasia. While most vulval intraepithelial neoplasia-related cancers occur in the first decade following diagnosis and treatment, later cases occur and, therefore, the patients need long-term follow up.

4 - The answer is false. It is the fourth most common.

5 - The answer is false. Vulval cancer is mostly a disease of older women but approximately 15% affect women under 40 years.

Question 2

1 - All patients should be referred to a gynaecological cancer centre for individualisation of treatment

2 - Women with lateral vulval lesions should have a radical vulvectomy and bilateral groin node dissection

3 - It is not necessary to remove the groin nodes in superficially invasive localised lesions

4 - Women with spread to the vagina are considered to have at least stage II disease

5 - Treatment of recurrence on the vulva is always palliative as the outcome is poor

Answer 2

1 - The answer is true. Improving Outcomes Guidance in Gynaecological Cancers recommends that treatment of rare cancers, such as vulval cancers, should be centralised in specialist cancer centres.

2 - The answer is false. In lateralised tumours, unilateral groin node dissection is sufficient.

3 - The answer is true. The risk of lymph node metastasis in tumours with a depth of invasion of less than 1 mm is minute and, therefore, node dissection is not warranted.

4 - The answer is true. A tumour of any size with extension to adjacent structures including the lower third of the vagina is considered stage II disease. A tumour of any size and extension with inguinofemoral lymph node metastasis is stage III disease. Extension into the upper two-thirds of the vagina is stage IV disease.

5 - The answer is false. Recurrent disease confined to the vulva can be treated successfully.

Question 3

1 - Radiotherapy should be considered in verrucous carcinoma of the vulva

2 - Radiotherapy should be considered in positive groin nodes

3 - Radiotherapy should be considered to allow sphincter preservation

4 - Radiotherapy should be considered in groin recurrences

5 - Radiotherapy should be considered in the primary lesion in womenunfit for surgery
.

Answer 3

1 - The answer is false. Anaplastic transformation has been recorded after radiotherapy for verrucous carcinomas, and therefore, radiotherapy should be avoided.

2 - The answer is true. This reduces the risk of groin recurrence in women with multiple positive nodes. Recurrence in the groin can be difficult to control and is extremely distressing for the woman.

3 - The answer is true. Preoperative radiotherapy to large tumours encroaching on the anal sphincter can be debulked with radiotherapy avoiding a potential anovulvectomy (and colostomy) to obtain clear surgical margins.

4 - The answer is true.

5 - The answer is true. Local control of disease can reduce unpleasant symptoms such as pain and discharge.

Question 4

Pathology of vulval cancers

Answer 4

• Approximately 90% of primary vulval cancers are squamous carcinomas.

Other histological subtypes of vulval cancer are:

malignant melanoma: 3%
basal cell carcinoma: 2–4%
Bartholin's gland tumour: 5%
adinocarcinoma: <1%
verrucous carcinoma: <1%
sarcomas: 1–2%.

Question 5

Risk factors for vulval cancer

Answer 5

• There are recognised predisposing factors to vulval cancer:
• 30% of squamous cancers are associated with high-risk subtypes of HPV VIN is increasing in incidence, particularly in women under 40 years of age, although the malignant potential of treated VIN is less than that of treated CIN at approximately 5%
• 30% of cancers develop in women with lichen sclerosus; the lifetime risk of developing cancer within lichen sclerosus is approximately 4%
• immunosuppressed women (e.g. transplant patients) are at higher risk
• Paget’s disease of the vulva is a rare condition and is regarded as an intraepidermal adenocarcinoma. Vulval adenocarcinoma may be present in 4–8% of women with Paget’s disease of the vulva.

Question 6

How do women with vulval cancer present?

Answer 6

The common symptoms and signs are:

pruritus (most common; 38–71% of patients)
bleeding
lump
ulceration
pain or burning
discharge
asymptomatic (up to 5% may be detected histologically in association with vulval intraepithelial neoplasia or carcinoma of the cervix or anus)
the typical appearance of a vulval cancer is of a raised ulcer with rolled edges.

Question 7

prognostic factors that influence survival in vulval cancer

Answer 7

There are five main prognostic factors that influence survival:
inguinofemoral lymph node metastases
FIGO stage
histological grade of the tumour
depth of invasion
age and performance status of the patient.
Metastatic disease in the regional inguinofemora

Groin node status	5-year survival (%)
Negative	92
Positive	
- Ipsilateral	75
- Bilateral	        30
- >2 nodes	25
- >6 nodes	0

Question 8

5-year survival rates in vulval cancer

Answer 8

The 5-year survival rates according to FIGO (1988) staging of vulval cancer.

Stage	5-year survival (%)
I	98
II	85
III	74
IV	31
Involvement of groin lymph nodes is the single most important prognostic factor that affects 5-year survival. Patients with negative groin nodes (all stages) have a 5-year survival of >90%. This falls to 52% in patients with positive groin nodes and 11% in patients with positive pelvic lymph nodes

Question 9

Key points in vulval cancer

Answer 9

The most important prognostic factor in vulval cancer is groin lymph node involvement.
Depth of invasion of the tumour is an important predictor of groin node involvement.
Tumour size and location are also predictive of groin node metastases.

Question 10

What investigations are required in vulval cancer ?

Answer 10

The diagnosis of vulval cancer should be confirmed by examination and biopsy prior to definitive surgical management. This may be carried out in the clinic under local anaesthetic.

Diagnostic biopsy should include the area of skin where there is a transition from normal to abnormal epithelium. Excision of the lesion should be avoided where possible until the diagnosis is obtained. It should be of sufficient size (depth of more than 1mm) to allow differentiation between superficially invasive and frankly invasive tumours.

The following information should be documented during examination for the purpose of treatment planning

size of the lesion
location of the lesion
appearance of background skin
involvement of vagina, urethra, bladder or anus
deep infiltration of pubic or ischial bones
presence of absence of enlarged groin lymph nodes.
Pre-operative imaging (CT or MRI) should be performed to exclude lymph node involvement and distant metastases. Chest imaging should also be performed pre-operatively (Chest x-ray or CT).

All cases must be reviewed by a specialist multi-disciplinary team prior to definitive treatment.

Question 11

Key points in managemnt of vulval cancer

Answer 11

Surgery is the mainstay of treatment for patients with vulval cancer.
Radical wide local excision of the primary tumour with a minimum margin of 15mm of normal tissue is sufficient.
Groin lymphadenectomy should be performed through separate incisions (triple incision technique) to reduce morbidity.
In lateralised tumours, ipsilateral groin lymphadenectomy can be performed initially.
In unifocal tumours less than 4cms in size, patients can be safely managed by removal of the identified sentinel lymph node.
Groin node dissection can be omitted in stage 1A tumours which are <2 cm in size with a depth of invasion < 1 mm

Question 12

Key points in adjuvant treatment of vulval cancer

Answer 12

Adjuvant radiotherapy should be considered in women with positive inguinofemoral nodes and in those with involved surgical margins.
Treatment should be to the groin and pelvic nodes.

Question 13

Advanced vulval cancer

Answer 13

• This is defined as stage III or IV.
• Treatment should be individualised based on patient’s performance status taking patient’s wishes into consideration.
• Multidisciplinary assessment of patient involving plastic surgeons, urologists, colorectal surgeons and clinical oncologists may be required to plan the appropriate treatment.
• Treatment options
• • Ultraradical surgery (radical vulval excision with partial or total exenteration and groin lymphadenectomy) with plastic reconstruction. This is associated with significant physical and psychological morbidity and postoperative mortality rates of 0–20%.
• • The use of preoperative radiotherapy & chemotherapy may shrink the tumour to allow less destructive surgery, in particular, preservation of sphincters and avoidance of stomas. Complete responses have been described using concurrent radiotherapy and a regimen of cisplatin and 5-flurouracil (5-FU). Combination of chemoradiotherapy & surgery is associated with significantly more morbidity than either treatment on its own.
• • Neoadjuvant chemotherapy with cisplatin and 5-FU followed by surgery or radiotherapy is an alternative treatment regimen, especially in those patients who have had perineal radiotherapy previously.
• • Primary radiotherapy with or without chemotherapy in those patients who are medically unfit for surgery.
• • Palliative treatment alone.

Question 14

Basal cell carcinoma and verrucous carcinoma

Answer 14

• These carcinomas are rarely associated with groin node metastases and are managed by wide local excision.
• Anaplastic transformation has been reported with radiotherapy in verrucous carcinoma and is, therefore, contraindicated. However, radiotherapy can be used in basal cell carcinomas and is the preferred mode of treatment if surgery is likely to compromise function.

Question 15

Malignant melanomas

Answer 15

• Wide local excision is only required where there is no evidence of benefit of groin node dissection.
• Prognosis depends on size of lesion and depth of invasion, usually using Breslow’s classification.

Question 16

Carcinoma of the Bartholin’s gland

Answer 16

This may be an adenocarcinoma or a squamous carcinoma. Treatment is similar to that of squamous carcinoma of the vulva. Lesions are usually deep-seated and resection may compromise the anal sphincter. Therefore, partial resection with reconstruction and a temporary defunctioning colostomy may be required. Women with a persisting Bartholin’s abscess or cyst should have a biopsy taken to rule-out carcinoma of the Bartholin’s gland (RCOG, 2014 (link is external)).

Question 17

Sarcomas of vulva

Answer 17

• Sarcomas represent 1–2% of all vulval tumours.
• Leiomyosarcomas are the commonest and present as an enlarging painful vulval mass.
• Treatment is wide local excision with or without adjuvant radiotherapy

Question 18

Key points in non squamous vulval cancer

Answer 18

• Groin node dissection can be omitted in patients with verrucous carcinoma, basal cell carcinoma and melanoma.
• Women with persistent Bartholin cyst or abscess should be suspected of having a possible carcinoma and biopsy should be taken.

Question 19

Follow-up of vulval cancer

Answer 19

Of all recurrences, 80% occur within the first 2 years. Follow-up is long term and should be in an environment where trained personnel are available to detect the earliest signs of recurrence. Follow-up interval should be based on local network guidelines but the RCOG recommends the following schedule:

3-monthly for the first year
6-monthly for the second year
annually thereafter.

Key points

Despite satisfactory primary treatment, up to a third of vulval cancers will recur.
Follow up may be required for many years and should be undertaken where trained personnel are available to detect early recurrence.
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Question 20

summary of vulval cancer

Answer 20

• Vulval cancer is one of the rarest gynaecological cancers.
• 90% of primary vulval cancers are squamous carcinomas.
• Risk factors include HPV, VIN, lichen sclerosus and immunosuppression.
• The most important factor determining survival is metastatic disease in the regional inguinofemoral lymph nodes.
• Management of women by multidisciplinary teams has made individualisation of treatment possible, so minimising mutilation of the vulva.
• In unifocal tumours of less than 4 cm in diameter where there is no clinical suspicion of groin node involvement, women can be safely managed by removal of identified sentinel lymph nodes.
• Reconstructive surgery has a very important role in the management of vulval cancer.
• Clinicians should be aware of psychosexual sequelae of vulval surgery and address this preoperatively.

Question 21

SBA 1

A 75-year-old woman presents via an urgent referral with a 4-month history of vulval pain. On examination she has a 4-cm irregular exophytic tumour replacing the clitoris and extending to within 15 mm of the urethra. Enlarged lymph nodes are palpable in both groins.

What is the next most appropriate action?

• Perform CT scan of the abdomen and pelvis
• Simple vulvectomy
• Urgent radiotherapy
• Vulvectomy & bilateral inguinofemoral lymphadenectomy
• Wedge biopsy of the lesion

Answer 21

Wedge biopsy of the lesion
The correct answer is wedge biopsy of the lesion. Although the clinical picture is strongly suggestive of vulval malignancy, a diagnostic biopsy is essential. A wedge biopsy will provide information regarding the type, grade and depth of invasion of the tumour. This information, in combination with imaging and MDT discussion, will facilitate appropriate treatment. In some cases, for instance basal cell carcinomas and verrucous carcinomas, lymphadenectomy is not necessary.

Question 22

SBA 2

A 75-year-old woman is seen in a urogynaecology clinic complaining of pelvic organ prolapse. Of note there is a history of intermittent steroid cream use for a sore and itching vulva. On examination there is a warty lesion on the vulva that is 1 x 1 cm on the left inferior aspect of the labia major. 

What would be the most appropriate action?

• Arrange excision biopsy of the entire lesion under local anaesthesia
• Prescribe topical imiquimod on the lesion
• Reassure the woman that vulval condyloma are common in postmenopausal women and concentrate on the reason for the referral, the pelvic organ prolapse
• Refer to a dedicated gynaecological onclogy/vulva clinic for an opinion
• Try a course of topical steroid and review the lesion in 6 months

Answer 22

Refer to a dedicated gynaecological onclogy/vulva clinic for an opinion

The correct answer is refer to a dedicated gynaecological onclogy/vulva clinic for an opinion. The management of vulval cancer ( RCOG 2006 ) states that suspected vulval malignancies should be managed by a dedicated vulval clinic or gynaecological oncologist and that newly acquired condylomas are unusual in this age group. In addition, if biopsy were performed, the lesion should be identifiable post biopsy (i.e. a wedge biopsy rather than excisional procedure).

The other options are incorrect because if the lesion is removed and the excision is done locally, the woman may need repeat surgery/will not remain suitable for sentinel node biopsies, which can have impact on her overall treatment. An incisional biopsy can be taken, but it is not an option that has been listed.

Question 23

SBA 3

A 77-year-old woman is seen by the GP for routine recall of her lichen sclerosus. She mentions the itching has become gradually worse over the year with no relief after using different creams as suggested by the pharmacist. She also mentions she has some swelling down below that feels like the size of a 10 pence coin. She is referred to the hospital and on examination, you note a raised ulcer with rolled edges. Her general examination is normal with no groin nodes palpable. A biopsy is taken under local anaesthetic in clinic. In anticipation, a pre-op is arranged.

Which of the following test is the most suitable for her?

FBC
FBC, serum biochemistry
FBC, serum biochemistry, CXR
FBC, serum biochemistry, CXR, ECG
FBC, serum biochemistry, CXR, ECG, CT pelvis

Answer 23

FBC, serum biochemistry, CXR, ECG
The correct answer is FBC, Serum biochemistry, CXR, ECG. This woman is very likely to have vulval carcinoma. 30% of cancers develop in women with lichen sclerosus with a lifetime risk of developing cancer within lichen sclerosus is 3–5%. In lesions that are 2 cm or less, a wide local excision biopsy is appropriate but should include a surrounding 1 cm zone of normal tissue. This can be performed under general anaesthetic. Vulval cancer patients are generally elderly and often have comorbidity so a preoperative anaesthetic assessment can be invaluable. Preoperative investigations should include FBC, serum biochemistry, CXR and ECG. Women with advanced disease should have a CT scan of the pelvis to exclude pelvic nodal involvement, which does not appear to be the case in this woman.

Question 24

SBA 4

You are about to see a 60-year-old woman at the vulva clinic. She initially presented 2 weeks ago complaining of soreness and irritation of the vulva. On examination there was is a 4.5-cm ulcerated lesion of the left anterior vulva, involving the clitoris. A punch biopsy was taken and the histology reveals a squamous cell carcinoma.

In addition to radical excision of the primary lesion, how will you counsel her regarding lymph node management?

Bilateral inguino-femoral lymphadenectomy is recommended
Lymphadenectomy is not required for this lesion
Sentinel node biopsy is recommended
Unilateral (left) inguino-femoral lymphadenectomy is recommended
Unilateral (right) inguino-femoral lymphadenectomy is recommended

Answer 24

Bilateral inguino-femoral lymphadenectomy is recommended
The correct answer is ilateral inguino-femoral lymphadenectomy. The RCOG/BGCS guidance recommends bilateral groin node investigation in lesions within 1 cm of the midline. The diameter of this lesion is above that recommended as safe (4 cm) for sentinel node assessment only.

Question 25

SBA 5

A 65-year-old lady was referred with soreness of the vulva. On examination, there is a raised irregular lesion with a erosive appearance measuring 1cm in diameter. What would you do next?

• Refer to a cancer centre
• Obtain a representative incisional biopsy
• Request MRI
• Perform a wide local excision
• Request a groin ultrasound scan.

Answer 25

Obtain a representative incisional biopsy

Any change in the vulval epithelium in postmenopausal women warrants a biopsy. Lesions should be biopsied rather than excised in order to allow for the identification and removal of sentinel nodes. A representative biopsy which includes the transition from abnormal to normal epithelium should be obtained for diagnosis.

Question 26

SBA 6

A 70-year-old lady presents with a 3.5 cm x 4.5cm raised irregular fungating mass on the left labia extending to the clitoris. A representative biopsy of this lesion has confirmed a moderately differentiated squamous cell carcinoma of the vulva with a depth of invasion of 8 mm. What is the most optimal management plan for this lady?

• Radical wide local excision alone
• Radical wide local excision and left groin sentinel node biopsy
• Radical wide local excision and left groin lymphadenectomy
• Radical wide local excision and bilateral groin sentinel node biopsy
• Radical wide local excision and bilateral groin lymphadenectomy.

Answer 26

Radical wide local excision and bilateral groin lymphadenectomy.

Excision of the primary tumour should be radical enough to obtain a 15 mm margin on all sides in a fresh surgical specimen. Dissection of groin nodes should be performed when the lesion has a depth of invasion greater than 1 mm or the maximum tumour size is more than 2 cms. Identification and removal of sentinel lymph nodes is safe only in patients with unifocal tumours less than 4 cms in maximum dimension. In patients with lesions greater than 4 cms, groin lymphadenectomy should be performed. As this tumour involves the midline (clitoris), bilateral groin lymphadenectomy should be performed.

Question 27

Stage I

At this stage, how would this cancer be managed?

Answer 27

Wide local excision

A small lateral lesion and wide local excision may be all that is required. The histopathologist would look at the depth of invasion of the specimen and if it is more than 1 mm, the benefits of ipsilateral groin node dissection would be discussed with the patient.

Question 28

At least stage II

The cancer is at least stage II. This is a large tumour extending at least into the lower third of the urethra and vagina.
How could it be managed?

Answer 28

Radiotherapy and/or chemotherapy

Imaging of the pelvis with MRI would be useful to assess local structures and inguinofemoral and pelvic lymphadenopathy. Radical surgery would jeopardise urinary continence so a multidisciplinary discussion regarding the use of radiotherapy and chemotherapy to shrink the tumour is essential.

Question 29

A 62-year-old woman presents to Miss Smith, a consultant obstetrician gynaecologist, with an itchy lump on the vulva. She has had this for three months and has noticed blood on her underwear for the past two weeks. She has no significant past medical history.

How could Miss Smith manage this woman?

Answer 29

full history and examination, including smear history, examination of the vulva, vagina and cervix
note position and size of the lesion and palpate for groin nodes
take biopsy (and smear if not up to date).

Question 30

A 62-year-old woman presents to Miss Smith, a consultant obstetrician gynaecologist, with an itchy lump on the vulva. She has had this for three months and has noticed blood on her underwear for the past two weeks. She has no significant past medical history.

The biopsy confirms a squamous cell carcinoma and Miss Smith reviews the woman at her next clinic.

What information would you give to the woman?

Answer 30

explanation of biopsy results (i.e. this is a cancer and she needs to be referred to a specialist gynaecological oncologist for treatment)
the likely nature of treatment – surgery to remove the lump and possibly removal of the nodes in the groins
that the aim of treatment is a cure
possible side effects of the surgery.

Question 31

Case study 4

A 77-year-old woman presents to her GP with a history of a sore, itchy vulva ‘for years’. She has well controlled NIDDM and glaucoma but is otherwise fit and well. The GP examines her and notices a suspicious lesion above the clitoris and refers her to a gynaecological oncologist.

At the gynaecological oncology clinic she is noted to have a central 5 × 4 cm ulcerated lesion above the clitoris. The surrounding skin is red with white plaques consistent with lichen sclerosis. There are no palpable groin nodes. A biopsy confirms a squamous cell carcinoma to a depth of 1.7 mm.
What is the appropriate surgery for this woman?

Answer 31

Wide local excision and bilateral inguinofemoral node dissection

The answer is wide local excision and bilateral inguinofemoral node dissection.

Question 32

Part 2

The woman in 4.1 has a stage II vulval cancer and a radical vulvectomy and bilateral groin node dissection is performed. Unfortunately, she develops troublesome lymphoedema.

At a routine clinic review 4 years later she is found to have a 1-cm warty lesion on the left posterior vulva.

What is your management now?

Answer 32

The answer is wide local excision.

Question 33

Part 3

This lesion described in part 2 of this case study is highly suspicious of recurrence but would be amenable to curative excision. A biopsy is taken to confirm the diagnosis.

A wide local excision is performed to include a 2-cm margin of normal tissue, but because of her previous surgery this is done using a rotational flap to cover the defect. The histology confirms a completely excised squamous cell carcinoma.

Some 2 years later, a 3 × 2-cm lesion is found at the left introitus. A biopsy confirms recurrence of the squamous cell carcinoma.

How would you manage this recurrence?

Answer 33

The answer is wide local excision.

A wide radical excision was performed with a rotational flap. The histology showed a poorly differentiated squamous cell carcinoma with clear margins and a depth of 6.2 mm.

If the woman is still fit enough for surgery and the lesion is amenable to further wide local excision, this should be performed.

Radiotherapy is another option but it has to be borne in mind that these recurrences are becoming closer together in time and it is likely that she will get a further recurrence.

It may be prudent to keep radiotherapy in reserve for the future.