Vulval cancer – an update TOG 2013

What is the Incidence?

 Vulval cancer remains a relatively rare condition accounting for 3–5% of female genital
cancers and historically affecting older women.The incidence of vulval cancer in young
women is rising.More than a 10‐fold increased incidence of cases in women younger
than 50 years of age over a 20‐year period.1

What is the most common Type?

 Approximately 90% of tumours are squamous in origin.

What is the Etiology?

 Exact aetiology unknown.

 First, tumours developing from vulval intraepithelial neoplasia (VIN) caused by human
papilloma virus (HPV) infection.
 Secondly, the development of squamous cell hyperplasia and atypia from chronic
inflammation and itch in non‐neoplastic vulval dermatoses such as lichen sclerosus. This
leads to HPV‐negative VIN and eventually to invasive keratinising squamous cell
carcinoma.
 HPV serotypes 16 and 18 are strongly associated with VIN.

What Presentation?

 Women with vulval cancer are rarely asymptomatic.

 The most common symptoms include pruritis, burning, soreness, bleeding, pain or a
lump.
 Genital warts are not common in postmenopausal women and so this finding should
raise the suspicion of cancer.
 Other concerning features are ulcers, a fungating mass, irregularity of skin contour,
depigmentation or hyperpigmentation and groin lymphadenopathy.
 Most squamous cell carcinomas are unifocal and occur on the labia majora .
 Other sites include the clitoris and perineum.

How the Diagnosis is made?

  Made by biopsy of a suspicious lesion.
 If the lesion is small and well circumscribed it may be possible to perform a wide local
excisional biopsy.
 It is preferable to use clinical photography to have a precise record of the site of the
lesion prior to performing the excision.
 A clearance margin of at least 1 cm, including depth, is required.
 If there is no wide margin of normal tissue then the lesion should be biopsied rather than
excised.
 Caution must be exercised when performing excision biopsies. If further surgery is
necessary, wide local excision can be extremely challenging when the initial lesion is no
longer present.
 For most lesions, it will be more appropriate to take one or multiple biopsies.
 The procedure can be performed with local anaesthetic using a Keye's biopsy blade.
 The site of biopsy is crucial; it should be taken from the edge of lesion and thus will
include adjacent normal epithelium.
 Plain lignocaine with or without adrenaline is first instilled with a fine needle.
 A punch biopsy is then driven full thickness through the epithelial surface.
 The surrounding skin is pressed to release the desired tissue.
 A soluble suture is usually required to achieve haemostasis.
 How the vulval ca spread ?
 The natural history of vulval cancer is to grow by direct extension
 followed by lymphatic embolisation.
 Initially this is to local inguinal lymph nodes and
 later to femoral
 and the external iliac chain.
 Final spread to distant sites is haematogenous.
 Lymph node involvement can occur early in the disease process.

What is the Staging of cancer of the vulva?

(adapted from FIGO Committee of Gynaecology Oncology), 2009.
Stage I Tumour 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.

Stage Tumour of any size with extension to adjacent perineal structures (1/3 lower
II urethra, 1/3 lower vagina, anus) with negative nodes.

Stage Tumour of any size, with or without extension to adjacent perineal structures, with
III positive inguino‐femoral lymph nodes.

IIIA (i) With 1 lymph node metastasis (≥5 mm), or

(ii) 1–2 lymph node metastases (<5 mm).

IIIB (i) With 2 or more lymph node metastases (≥5 mm), or

(ii) 3 or more lymph node metastases (<5 mm).

IIIC Positive nodes with extracapsular spread.

Stage Tumour invading other regional (2/3 upper urethra, 2/3 upper vagina), or distant
IV structures.
 Stage I Tumour confined to the vulva.

IVA Tumour invades any of the following:

(i) upper urethral and/or vaginal mucosa, bladder mucosa, rectal mucosa, or fixed
to pelvic bone, or

(ii) fixed or ulcerated inguino‐femoral lymph nodes.

IVB Any distant metastasis including pelvic lymph nodes.

 What are the survival rates?

 Mortality is directly related to stage of disease at presentation.
 Data from the National Cancer Institute shows
 a 5year survival rate of 86% for stage I
 53% for stage III
 15% for those with distant metastases.

 In cases where there is a high suspicion of malignancy, a multidisciplinary approach is

required taking into account the woman's age, gestation, parity, desire for fertility and the
likely stage of disease.

 Pathological examination will confirm histological type of cancer and give details of depth
of invasion and clearance margins.

 Depth of invasion is defined as the measurement from the epithelial junction of the most
superficial adjacent dermal papillae to the deepest point of invasion. It directly correlates
with lymph node involvement.

 Tumours with less than 1 mm invasion have a negligible (less than 1%) risk of lymph
node metastasis thus the need for nodal resection is eliminated in these cases. These
tumours are termed superficially invasive squamous carcinoma.
  All other squamous vulval cancers have a risk of nodal spread that is directly correlated
to depth of invasion.

 What is verrucous ca?

 The exception to the rule is verrucous carcinoma, a subtype of squamous carcinoma.

These typically large condylomatous lesions are well differentiated, low grade fungating
tumours and do not metastasise.

What is the Treatment?

 Vulval cancer should be managed by a multidisciplinary team in a cancer centre.
 Surgery remains the gold standard of treatment for vulval cancer.
 Treatment should be tailored to the individual and should take into consideration her
age, fitness, sexual function, tumour size, tumour site and stage of disease.
 What type of anaesthesia?
 Although most commonly performed under general anaesthesia it should be
remembered that for those women deemed unfavourable for this mode of anaesthetic,
 a combination of regional anaesthetic with conscious sedation has been shown to be an
effective and safe alternative.

Treatment of early vulval cancer

 The current trend of management of early vulval cancer (stage II or less) is to leave as
much vulval tissue as possible.
 data found no difference in local recurrence rates between radical wide local excision
and radical vulvectomy.
 If the primary lesion is a superficially invasive squamous cell carcinoma then resection of
lymph nodes is not necessary.
 For all other tumours management includes inguinofemoral lymphadenectomy.
 Lateral tumours, defined by their location of more than 1 cm from the midline, drain to
the ipsilateral lymph nodes.
 For these tumours, performing an ipsilateral lymph node resection has the same
outcome with regard to groin recurrence as a bilateral resection. 
 If the lesion is central then both groins should be resected.
 Superficial lymph node resection results in a higher incidence of recurrence so both
superficial inguinal and deep femoral lymph nodes should be resected. 
 En bloc groin resection has been replaced with dissection through a separate incision.
 What Surgery related morbidity ?
  It is a substantial problem and is mainly related to groin resection.
 short‐term complications (wound breakdown, infection and lymphocele).
 Radiotherapy may be required as an adjuvant for early disease, for example in cases
where margins are inadequate (less than 8 mm) or if multiple lymph nodes display
microscopic involvement.

Treatment of advanced vulval cancer

 For stage III and IV disease a combination of treatment modalities can be used
according to the individual case.
 Surgery entails radical vulvectomy with en bloc resection of bilateral inguinofemoral
lymph nodes.
 Most patients require post operative radiotherapy to the pelvis and groin.
 Pre operative neoadjuvant radiotherapy or chemoradiation can be used to improve
operability and reduce extent of surgery required including those cases that would
otherwise require a stoma.
 Evidence supports the use of chemoradiation in cases of advanced disease
o that would otherwise warrant pelvic exenteration.

What is the sentinel lymph node?

 It is the node to which the tumour first drains.

 For women with early disease, only 20% will have lymph node metastases.
 If the sentinel lymph node is isolated and resected, it can be examined by frozen section
pathology.
 A sulphur colloid tagged with the radioactive technetium‐99 m is injected near the
tumour.
 By using scintigraphic imaging, the node or nodes that take up the radioactive substance
can be identified.
 Similarly, blue dye can be injected intraoperatively about 15 minutes before biopsy.
Visual inspection can then identify the sentinel node.
 A frozen section procedure takes less than 20 minutes enabling rapid microscopic
analysis of the node.
 If there is no evidence of metastases then further resection can be avoided.

What happened if Recurrence?

  For primary vulval carcinoma studies have shown a recurrence rate of 37%.
 More than half of the recurrences occurred at the perineum.
 Risk factors for recurrence
 FIGO stage greater than II
 Positive lymph nodes
 vascular space invasion
 Site of recurrence correlated to survival with
 a 5 year survival rate of 60% for perineal recurrence,
 27% for inguinal and pelvic recurrence and
 15% for distant recurrences.
 Radical re‐excision or radiation can be employed to manage local relapse.

What are the Pshycosexual Issues?

 It is essential to address the sexual impact that genital surgery can have.
 The issue should be raised pre operatively and ideally included in the consent process.
 Scarring and loss of genital architecture can cause narrowing of the introitus and, in
 turn, lead to dyspareunia.
 It can also leave a woman feeling defeminized.
 Arousal and sexual pleasure can be diminished and it may not be possible to achieve an
orgasm.
 It is common for women to feel numbness in the genital area following vulvectomy
although this may improve with time.
 Estrogen after radiotherapy was suggested to have short‐term benefit
 A psychosexual counsellor and reconstructive plastic surgeon have an important role is
managing sexual issues and should be part of the multidisciplinary team caring for
women with vulvar cancer.

What about Reconstructive Surgery?

 Reconstructive surgery plays an important role in the cosmetic and functional results of
wide radical vulval surgery.
 Gynaecological oncologists will have experience in such surgery but input from a plastic
reconstructive surgeon is often necessary.
 Surgical techniques range from simple procedures for introital stenosis to complicated
procedures where large areas of skin and underlying tissue are used as flaps to cover
defects caused by radical surgery.
  It is preferable to perform reconstruction at the same time as primary surgery.
 A variety of graft procedures, realignment of standard incisions and use of vascular
pedicle flaps can be employed.
 Use of fasciocutaneous or and myocutaneous skin flaps for defects led to excellent
wound healing and cosmetic results.
 Reconstruction can allow for more radical excisions and thus the achievement of
sufficient clearance margins can be improved.

 There can be a role for reconstructive surgery at a later stage.

 Cosmetic appearance of the vulva can be enhanced by procedures such as labial
reconstruction.

Conclusion

 Vulval cancer is rare and so should be managed in specialist cancer centres with a
multidisciplinary approach.
 The principle of management is to eradicate disease whilst preserving as much function
as possible.
 Morbidity may be reduced by the use of sentinel node biopsy although this treatment
should be undertaken in the context of a clinical trial until further research data are
available.
 Sexual dysfunction is an important and distressing consequence of vulvar surgery that
needs to
 be recognised by health professionals and addressed with patients before surgery.

Prepared By

Dr Fariha Altaf