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Vulval Cancers

by

Thomas Ind

Consultant Gynaecological Surgeon

St George's & Royal Marsden Hospitals

 

Introduction

90% of all vulval malignancies are squamous

3.5% are malignant melanoma

The rest consist of;

        Basal cell carcinoma

        Sarcoma (e.g. botryoides)

        Adenocarcinoma

        Secondary cancer

 

Squamous Cell Carcinoma of the Vulva

Incidence

·        Low

·        Approximately 800 new cases registered in UK annually

·        Mortality in 1996 was 340 cases

·        Death rate of 1.29 per 100,000 women

·        3% of all genital cancers

Age

·        Old age

·        Mean age in own series is 71 years

Geographic

More common in Western World

 

Aetiology

·        Epidemiological

·        Microbiological

·        Immunological

·        Molecular

EPIDEMIOLOGY

·        Obese, hypertensive, diabetic and nulliparous (RUBBISH)

·        Association with cervical cancer

·        Multiple sexual partners

·        Smoking

·        History of genital warts

MICROBIOLOGY

·        HPV

o       60% of patients

o       Younger group of women

o       VIN often associated with invasive lesion

·        Granulomatous veneral disease (Jamaican study found 66%)

o       Syphilis

o       Lymphogranuloma venerum

o       Granuloma inguinale

·        Granulomatous associated disease is probably different to that seen in UK

 

IMMUNOLOGICAL

·        Loss of skin barrier

o       Erythema

o       Lichen sclerosis

·        Cell mediated immunity

o       Antigen presenting cells

o       Lymphcytes

o       Interleukins 1 & 2

MOLECULAR

·        E6/E7 – P52 apoptotic regulation

Spread

 

Local spread

        Tumour <2cm                 T1     FIGO Stage 1

        Tumor 2cm or more         T2     FIGO Stage 2

Urethra                          T2     FIGO Stage 2

        Vagina                           T3     FIGO Stage 3

        Anus                              T3     FIGO Stage 3

        Thigh                             T3     FIGO Stage 3

        Abdomen                        T3     FIGO Stage 3

        Upper urethra                  T4     FIGO Stage 4

        Bladder                           T4     FIGO Stage 4

        Rectal mucosa                  T4     FIGO Stage 4

 

Lymph glands

        Unilateral                         N1    FIGO Stage 3

        Bilateral                           N2    FIGO Stage 4

 

 

Lymphatic spread is to inguinal  nodes and then femoral nodes although it is well reported that femoral nodes can be involved without superficial nodal involvement (direct spread).

 

The pelvic nodes can be involved. This is most commonly external iliac
involvement with spread from the femoral node. However, other pelvic nodes can be involved if the tumour has spread to the upper part of the vagina. Bladder or rectum

 

Contralateral nodal involvement is rare in truly lateral lesions (i.e. those which are greater than 1cm from any midline structure).

 

There are problems with the FIGO staging system. The 1968 system is that mis-quoted by the RCOG “clinical recommendations for the management of vulval cancer” (1999). Most data available is from the clinical staging. However, this is poor as 10% of women with clinical stage 1 disease will have positive lymph nodes. This led to the 1988 surgical staging. Patients with negative nodes fair well irrespective of the size of the primary tumor. The TNM classification is therefore preferred by most sub-specialists in gynaecological oncology.

 

FIGO Clinical Staging (1968)

 

Revised FIGO staging 1988

 

Lymph node positivity rate according to 1968 staging

 

  

Survival according to 1988 stage classification

 

  

Treatment

 

RCOG and NHS recommendations are that patients with proven vulval cancer should be treated in a cancer centre.

 

Must be set routes and protocols for referral and diagnosis in cancer units for women with suspicious vulvas to allow adequate staging and prevent delay.

 

Previously, treatment for SCC vulva consisted of a radical vulvectomy, bilateral inguino-femoral lymphadenectomy and pelvic lymphadenectomy through a ‘butterfly’ incision. Now treatment is modified to reduce the complications.

 

All patients should have a full EUA including representative biopsies and a colposcopy and vaginoscopy due to a strong association with cervical and vaginal cancer and precancer.

 

Example of pathway for treating SCC of the vulva

 

 

Old butterfly incision for treating vulval cancer

 

 

New(er) triple incision

 

  

It is now known that a triple incision reduces the complication rates for vulval cancer without decreasing the survival. Other methods of conservative surgery which are either proven or being evaluated include;

o       Saphenous sparing groin dissection

o       Short incisions for groin dissection (<8cm)

o       Assessment with groin ultrasound and fine needle aspiration with aim of abandoning the procedure if negative

o       Sentinal node dissection using dye and radiolabel to determine if groin dissection is required

A number of studies have now demonstrated that for unifocal T1 lesions, a radical local excision is as efficacious as a radical vulvectomy.

 A radical local excision goes to the deep fascia in a similar way to a radical vulvectomy and ensures that there is atleast a 1cm margin from the tumor. (A simple vulvectomy is a treatment for VIN and only requires a superficial excision). Other names for a radical local excision include a ‘Wide local excision’, a ‘Hemivulvectomy’, and a ‘Partial vulvectomy’.

 

The prognosis is also affected by the depth of invasion. The depth of invasion influences the lymph node positivity rate.

 

Lymph node positivity is stage 1 SCC of the vulva

 

 

As a result of this, it is unnecessary to perform a lymphadenectomy if the depth of invasion is less than 1mm.

 

The incidence of contra-lateral node involvement for truly lateral lesions is <0.5%. Therefore, the consensus is that women with truly lateral lesions should not have a contralateral lymphadenectomy unless there is histologically proven ipsilateral node involvement. (A second operation required).

 

The prognosis is also affected by the number of positive lymph nodes and the extent of lymph node involvement (microscopic deposits, capsular involvement or complete replacement). The GOG study demonstrated that radiotherapy is of value in women with 2 or more nodes involved or one node with extra-capsular involvement. These patients get groin and pelvic radiotherapy.

 

As pelvic lymph node involvement occurs in <0.5% of women without groin nodes involve, pelvic lymphadenectomy is never necessary (as women with groin nodes get pelvic radiotherapy anyway).

 

Pathways for the management of women with a suspicious vulva

 

 

Pathways for the follow-up of women with SCC of the Vulva

 

 

There is no evidence that follow-up is of any benefit to a woman with vulval cancer in terms of follow-up. However, it has benefits in terms of data collection and reassurance. It does not matter who follows the woman up.

 

Imaging

There are number of studies underway looking at the value of MRI, lymphangiography, needle aspiration, radio-immunoscintigraphy and sentinal node dissection to determine lymph node positivity. At present, none of these studies have demonstrated a reliable method of lymph node assessment however, this may change soon with publication of ongoing studies

 

Basal Cell Carcinoma of the vulva

They account for about 2% of vulal cancer. They rarely metastasises to the lymph nodes and lymph node dissection is therefore not required. However if they contain a squamous element (basosquamous carcinoma) they should be treated like a SCC of the vulva.

 

Bartholin’s Gland Cancer

This accounts for about 5% of all vulval cancers and only about 250 cases have ever been reported. A variety of histological types can exist including adenocarcinoma, squamous carcinoma, adenosquamous and rarely transitional cell. The adenoid cystic variety has a better prognosis.

 

In about 10% of cases it is proceeded by a Bartholin’s abscess and the diagnosis may be delayed. It is for this reason that a biopsy must always be taken when treating a bartholin’s cyst or abscess.

 

To classify as a Bartholin’s gland cancer they have to fulfil Honan’s criteria;

        Correct anatomical position

        Tumor deep in labium majus

        Overlying skin intact

        Some recognisable normal gland intact

 

Vulval Melanoma

Incidence

Rare but the second most common vulval cancer. Even though the vulva is not exposed to the sun light, this area of skin is three times more likely to develop a melanoma than any other. The reason for this unclear. The incidence is increasing.

 

Age

Predominantly is post-menopausal women

 

Geographic

Western world

 

Predisposing factors

Unknown

 

Pathology

Three main types;

Superficial spreading – relatively superficial in early development

Lentigo maligna melanoma – flat freckle

Nodular melanoma – most aggressive

 

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