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VULVAL CARCINOMA
Index
1.
Clinical symptoms and signs
2.
Screening
3.
3.1
3.2
3.3
Referral pathway
For GP
For non-oncological consultants/ firms
For referral from unit to centre
4.
Diagnosis
5.
Pre-invasive disease
6.
6.1
Gynaecological cancer multidisciplinary team
Information
7.
Pathology
8.
Staging
9.
9.1
9.2
9.3
Histology reports
Punch biopsy
Small ellipse of skin
Excision biopsy
10.
Histopathology minimum dataset
11.
11.1
11.2
11.3
11.4
11.4.1
11.4.2
11.5
11.6
11.7
Treatment
Microinvasion
Lateral tumours
Radical vulvectomy
Other considerations
Exenteration
Sentinel node dissection
Adjuvant radiotherapy
Morbidity
Surgical management of non squamous vulval cancer
12.
Dealing with recurrent disease
13.
Treatment algorithms for patients with carcinoma of the vulva
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13.1
13.2
13.3
13.4
13.5
13.6
Primary therapy
Adjuvant therapy to the primary
Adjuvant therapy to the lower pelvic and inguinal nodes
Individualised therapy for patients with recurrent or metastatic disease
Pelvic exenteration
Palliative care (see Palliative care file)
14.
14.1
Survival
Cancer dataset/ inventory of active trials
15.
15.1
Follow up
Identification and management of late effects of treatment
16.
Contact names/ numbers
17.
Algorithm
18.
Summary
19.
References
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Introduction
This is a rare carcinoma accounting for only 3-5% of all genital tract carcinomas with 803 new cases in
1992 and 346 deaths in England and Wales in 1997 (ONS data). It is usually a disease of the elderly
woman. There may have been a long history of vulval irritation and pruritis. There may have been a
skin dystrophy. Vulval condyloma may be seen in association with carcinoma and human papilloma
virus may have a role in carcinogenesis. In younger women carcinoma of the vulva and vulvar
intraepithelial neoplasia are seen in association with other cancers and precancers of the genital tract.
The younger patient has a tendency to develop multifocal disease of the vulva and perianal region.
1.
Clinical symptoms and signs
Over 2/3 present with irritation and vulval pruritis and usually of long duration. Patients may notice a
lump or pain. Bleeding or discharge can also be reported. Tumours tend to affect the labia, the left more
than the right. The second commonest site being the clitoris.
As patients present in their 7th. decade there may be appreciable medical problems and these must be
corrected before major surgery is contemplated. However operability rates of 96% have been reported
in large centres (Podratz et al, 1982; Monaghan and Hammond, 1984).
2.
Screening
There is no screening procedure for vulval cancer. Patients with carcinoma of the vulva are at an
increased risk of developing other genital cancers, particularly cervical cancer.
3.
Referral pathway (see 16. Contact names and numbers)
3.1
For GP
If vulval cancer is suspected then referral should be to a general gynaecologist, the lead in the cancer
unit or gynaecological oncologist in the cancer centre.
Standards
Rapid access to the specialist should be available with the patient seen within 2
weeks of date of receipt of the referral letter/ fax.
Definitive treatment should be commenced no later than 62 days after receipt of the
referral letter/ fax.
Definitive treatment should be commenced no later than 31 days after diagnosis for
non urgent suspect cancer referrals.
3.2
For non-oncological consultants/ firms
If the diagnosis is suspected or confirmed referral to the local unit lead or gynaecological cancer centre
should be made.
3.3
For referral from unit to centre
This is appropriate for all cancer cases.
4.
Diagnosis
This depends upon examination under anaesthesia with biopsy. Particular attention is paid to the
proximity of the tumour to the external urethral meatus, anal verge, the size of the primary mass and its
fixity. Large tumours may occasionally require skin grafting to achieve primary closure.
A pre-operative chest X-ray is useful to detect occult extension of disease.
CT scanning of the abdomen and pelvis may be useful to assess occult pelvic extension for clinically
staged III or IV disease.
Fine needle aspiration of clinically suspicious nodes is recommended.
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5.
Pre-invasive disease
A number of types of intraepithelial malignancy and pre-malignant conditions affect the vulva.
Intraepithelial malignancies include vulval intraepithelial neoplasia of classical and so-called
differentiated type, vulval Paget’s disease and in-situ malignant melanomas. The proportion of these
in-situ malignancies that progress to invasion is difficult to accurately assess. It is reported to be up to
5% for VIN. Lichen sclerosus and epithelial hyperplasia are also suggested by some workers to be premalignant conditions. The pre-malignant potential of lichen sclerosus has been extensively debated over
the years but is still unclear. Vulval Paget’s disease may be associated with underlying invasive
adenocarcinoma, for example from adnexal structures (in up to 20% of cases) and also has an
association with adenocarcinomas at other primary sites (in 20-30% of cases).
Colposcopy is useful for localisation but the signs are non-specific. Squamous hyperplasia and VIN are
treated with local excision or vulvectomy with 1cm disease free margins. Vulval Paget’s disease is
managed similarly but requires a 2cm margin (Kurzl, 1993). Groin node dissection is inappropriate.
6.
Gynaecological cancer multidisciplinary team
Core team
Named team member
Gynae oncologist
Gynae lead cancer surgeon
Medical oncologist
Clinical Oncologist
Pathologist/ Cytopathologist
Palliative care team
Radiologist
MDT co-ordinator
CNS
Extended team
Mr Leeson / Mr Toon
Mr Bickerton/ tba
Prof Stuart
Dr Al-Sammarie
Dr Lord
Dr Williams
Dr Barwick
Ms Jones
Ms Hall
Ultrasonographer
tba
Junior doctors
Psychologist
Geneticist
Ms Grier
Social worker
tba
Ward Sister
Sr Williams
Research nurse
Colorectal/ urological/ plastics as required
6.1
Additional member or
Cover (core team only)
Dr Williams
tba
tba
tba
Dr Wenham
tba
Core/ extended teams to include
members from YGC/ NEWT
Information
Standards
All patients must have access to a gynaecological oncology clinical nurse specialist
within 24 hours of the patient being informed of her diagnosis (this should include a
daytime contact telephone number for the clinical nurse specialist). Preferably the
nurse specialist should be at the consultation when the patient is given her diagnosis.
All referring practitioners and/ or patients GP’s should be informed by letter or
secure fax within 24 hours of the patient being informed of her diagnosis.
All patients must be given appropriate literature about the management, treatment
and outcome for cervical cancer such as a Cancerbackup leaflet or equivalent.
All these activities must be documented in the patient’s case record.
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7.
Pathology
Most carcinomas are squamous (85%). Five percent are malignant melanomas and the remaining 10%
are made up of carcinomas of the Bartholin’s gland, sarcomas and others.
Squamous cell carcinomas include the rare verrucous carcinoma which is relatively slow growing. For
this latter carcinoma, wide local excision or simple vulvectomy is usually sufficient. For those patients
with squamous cell carcinoma then the degree of differentiation most closely predicts metastatic
potential. Overall squamous cell carcinoma has a 5 year survival of 94% (Monaghan and Hammond,
1984). The presence of infiltrative growth patterns, compared with a pushing pattern, is associated with
a higher local recurrence rate. Lymphovascular space involvement (LVSI) is also associated with an
increased local recurrence rate. LVSI has not been associated with a higher risk of groin node
metastasis. Both factors are markers of poor prognosis, but they do not indicate the need for adjuvant
therapy.
Melanomas can be melanotic or amelanotic and are extremely aggressive carcinomas. In subcutaneous
tumours (tumour thickness >3mm), there is a substantial risk of recurrent disease whatever operation is
chosen. En bloc dissection of groin nodes with the primary specimen does not offer a survival
advantage to that of wide local excision (DeMatos et al, 1998; grade B recommendation) for disease
involving deeper levels.
Carcinoma of the Bartholin’s gland represents only 1-3% of all vulval carcinomas. Such tumours
commonly involve groin and pelvic nodes with a correspondingly poor survival (52% at 5 years).
Sarcoma of the vulva is rare and has a tendency to local and blood borne spread. Surgery is often
combined with radiotherapy and chemotherapy.
Basal cell carcinoma is treated by local excision. Basal cell carcinoma, verrucous carcinoma and
superficially invasive carcinoma are very rarely or never associated with lymph node metastases.
8.
Staging
Stage I
a
b
Stage II
Stage III
Stage IV
a
b
All lesions confined to the vulva, maximum diameter 2cm
and no suspicious groin nodes
up to 1mm depth of invasion
more than 1mm depth of invasion
All lesions greater than 2cm in diameter and no suspicious groin nodes
Lesions extending beyond the vulva to lower vagina or anus
and/or unilateral regional lymph node metastases.
Lesions involving the rectal, bladder or urethral mucosa or bone
and/or bilateral regional lymph node metastases.
All cases with distant metastases including pelvic lymph nodes.
FIGO, 1988
9.
Histology reports
9.1
Punch biopsy
The report should describe the condition of the epidermis (e.g. acanthotic, hyperkeratotic,
parakeratotic), the presence or absence of features suggesting HPV infection (eg. koilocytes,
dyskeratotic cells, multinucleated cells), the presence or absence of CIN and its type (ie. differentiated
or classical), with grading where possible, and the presence or absence of invasive neoplasia with its
type. The report should make clear whether any neoplastic abnormality is squamous, glandular and
melanocytic and if any invasion is present, the depth of invasion in the available tissue should be stated
and the presence or absence of lymphovascular permeation indicated. It should be made clear in
reports where there is invasion when this involves the biopsy margins, which will be presumed to be the
case in the majority of tumours. The excision planes are clearly not of relevance in most punch
biopsies.
9.2
Small ellipse of skin
For small eclipses of skin, the content of the report would be similar to that for a punch biopsy, or a
small excision biopsy depending on the lesion size. It may be possible to state whether a lesion has been
completely excised if small.
9.3
Excision biopsy
As well as all the details listed in the punch biopsy report, the invasive tumour should be typed, and
graded in line with the protocol. The maximum diameter of the tumour should be measured, as should
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the maximum depth of invasion from the tip of the nearest adjacent overlying dermal papilla and the
clearance to each of the resection margins, including the deep margin, should be clearly stated. There
should be a clear comment on the completeness of excision, the tumour free margins and the presence
or absence of lymphovascular permeation. In those specimens which include node dissections, all
lymph nodes found should be fully examined and comment made on the presence or absence of
metastatic carcinoma, together with comment on whether there is extracapsular tumour spread. It should
be noted that excision specimens for VIN should be embedded and examined in their entirety in order
to define clinically silent areas of invasive carcinoma.
The total number of lymph nodes and the number containing metastases should be given, together with
details of any extracapsular spread and the number of nodes where this is seen.
10.
Histopathology minimum dataset
All reporting should follow the protocol laid down by the Royal College of Pathologists.
 The presence of absence of VIN and if present its grade.
 The presence or absence of invasion. If invasion is present, further details of the lesion should
be provided (see below).
 The adequacy of excision of the neoplastic lesion. This is not appropriate for punch biopsy
specimens.
 The presence of HPV associated features (koilocytosis, epithelial multinucleation, individual
cell keratinisation, parakeratosis, acanthosis and papillomatosis).
 The presence of lichen sclerosus and/ or squamous hyperplasia in the adjacent non-neoplastic
epitheliuium.
If invasion present the following details should be recorded
 Tumour type
 Tumour grade
 The size of the lesion
 The presence or absence of lymphovascular invasion
 The adequacy of excision
The histology report should include the following information
Gross description
 Site
 Size (in three dimensions)
Histological
 Associated features (eg. conylomata etc.)
 Tumour type
 Depth of invasion
 Maximum horizontal measurement
 Differentiation
 Whether tumour is completely excised
 Tumour free margin
 Vascular space invasion
 Nature of adjacent non-malignant squamous epithelium
 Lymph node status
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NATIONAL MINIMUM DATASET
VULVAL CANCER HISTOPATHOLOGY
Nature of Specimen:……………………………………………………………………………………………..
Gross description
Length ..........mm
Size of specimen:
Breadth ...…...mm
Depth ........mm
No residual tumour: 
Site(s) of tumour : …………………………………………………………………………………………………….
Dimensions of tumour:
Length ..........mm
Breadth ...…...mm
Depth ........mm
Histology
Histological type:
squamous (usual) 
basaloid 
other (please specify)  …………………
Histological differentiation:
Histological size:
well/moderate  poor 
maximum horizontal dimension ………….. (mm)
depth of invasion
..………... (mm)
Minimum tumour-free skin margin
………….. (mm)
Minimum tumour-free vaginal margin
………….. (mm)
Minimum tumour-free anal margin
………….. (mm)
Minimum deep soft tissue margin
………….. (mm)
present 
VIN :
N/A
N/A


absent 
Nature of adjacent non-neoplastic skin …………………………………………………………………………
Lichen sclerosus 
Lymphovascular invasion:
Squamous hyperplasia  HPV-associated features 
present 
absent 
Groin nodes: total number of nodes (right) ….…
total number of nodes (left) …….
total number of positive nodes (right) ….… total number of positive nodes (left) …….
Extranodal extension:
yes 
no 
Comments
SMOMED Codes
T80100 Vulva
T08000 Lymph node
North Wales Cancer Guidelines, Vulval Cancer (June, 2008)
M80703 Squamous cell carcinoma
M80416Metastatic squamous carcinoma
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11.
Treatment (see 17. Algorithm and 18. Summary)
11.1
Microinvasion
The concept of microinvasive disease applies in vulval carcinoma to lesions up to 1mm maximum depth
of penetration (classified as stage Ia disease). This enables a less radical procedure dispensing with
groin dissection providing histological examination excludes vascular space involvement and
confluence of tumour buds. The choice of resection is then between local excision and simple
vulvectomy (grade B recommendation). Hoffman et al, (1983) noted that 36% of tumours displaying
confluence had nodal metastases as opposed to none in those that did not have confluence. Hence a
considerable degree of individualisation can be incorporated into treatment for patients with carcinoma
of the vulva (see table 1).
Table 1
Depth of invasion (mm)
% positive groin nodes
<1
0
1.1 to 2
7.7
2.1 to 3
8.3
3.1 to 5
26.7
>5
34.2
11.2
Lateral tumours
For stage Ib/ II tumours at least 2cm lateral to the midline and lateral to the labium minus, ipsilateral
groin dissection can be considered for lesions without capillary space invasion or tumour confluence
providing that frozen section nodal examination at operation and subsequent definitive microscopic
nodal assessment shows no tumour metastases (Cavanagh and Hoffman, 1996; Stehman et al, 1992;
grade B recommendation). However if positive nodes are found here then the other groin must be
explored or radiotherapy offered. Central tumours have a predilection for bilateral spread to groin
nodes and bilateral lymphadenectomy should be routinely performed.
11.3
Radical vulvectomy
The standard operation is radical vulvectomy. This involves removal of the vulva extending from the
vaginal introitus to the outer borders of the labia majora and removal of the superficial and deep
inguinal (groin) nodes either en bloc with the primary tumour as a ‘butterfly’ incision or with a triple
incision where the groin nodes are removed separately. The excision should have a 2cm disease free
margin around the primary tumour. 8mm is usually taken as the minimum margin for not prescribing
radiotherapy (RCOG 1999; de Hullu et al, 2002: see table 2). The triple incision approach is generally
preferred as it produces a less disfiguring scar and less morbidity for no statistically significant
reduction of survival (Magrina et al, 1998; Rodolakis et al, 2000; de Hullu et al, 2002: evidence level
3; grade B recommendation). It is better reserved for smaller tumours (stage I and II) where
micrometastases within the skin bridge are unlikely. In cases with a large primary tumour and clinically
suspicious nodes a radical vulvectomy with en bloc dissection may be warranted (grade B
recommendation). Patients with fungating groin nodes should have the groin dissection performed in
continuity with the vulva.
Table 2
Disease free margin (mm)
Local recurrence risk (%)
<4.8
54
4.8 to 8
>8
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Suction drainage is provided to the groins, prophylactic antibiotics and prophylaxis against
thromboembolism are routine.
If possible the great saphenous vein should be preserved as this is associated with less lower limb
cellullitis, groin wound breakdown and chronic lymphoedema of the lower limb (Zhang et al, 2000;
evidence level 3; grade B recommendation). A greater metastatic potential has been noted for primary
tumours more than 4cm in diameter. Pelvic node dissection is not of any use on a routine basis. Even
when the groin nodes are heavily infiltrated then salvage with pelvic node dissection in pelvic node
positive patients is poor. If the patient has distant metastases then radical vulvectomy still allows the
patient to escape the misery of death from untreated vulval disease.
11.4
Other considerations
11.4.1 Exenteration
Tumours involving the bladder, rectum, anus or vagina require a more radical approach involving some
form of exenterative procedure. If the tumour invades bone then a segment of the pelvis can be
removed, the patient having normal mobility. However tumour bulk can be reduced with pre-operative
chemoradiation with concurrent 5FU to the vulva and groins (Wahlen et al, 1995) thereby reducing the
radicality of subsequent surgery.
Patients are to be referred to Liverpool Womens Hospital if exenteration to be considered and
all cases must be discussed at the gynae cancer MDT to decide further treatment.
11.4.2 Sentinel node dissection
Technetium labelled sentinel node dissection appears a reliable technique to indicate which patients
with stage Ib/ II disease may be spared groin dissection with a negative predictive value approaching
100% for a negative sentinel node to be associated with no groin metastases. At present this is a
research tool and not part of routine clinical practice until supported by a randomised controlled clinical
trial (Moore et al, 2005). Patent or isosulfan blue dye appears unreliable to identify sentinel lymph
nodes for vulvar carcinoma (Ansink et al, 1999; Moore et al, 2003).
11.5
Adjuvant radiotherapy
A survival advantage has been noted for patients given groin radiotherapy if more than one inguinal
node contained tumour at primary surgery (Homesley et al, 1986; grade A recommendation), if there is
extracapsular spread in any lymph node or if tumour extends to within 8mm of a surgical margin (Heaps
et al, 1990; grade B recommendation). In cases with fixed or ulcerated groin nodes radiotherapy to the
affected groin and pelvic nodes should be considered rather than surgery (RCOG, 1999; grade C
recommendation). Pathological assessment of these nodes should be undertaken prior to radiotherapy
either by open biopsy or by fine needle aspiration cytology.
Standards
Radiotherapy should start 14 days after referral for radical treatment (good practice)
although 28 days is acceptable (minimum standard).
Radiotherapy should start 28 days after referral for adjuvant treatment (minimum
standard).
JCCO/RCR guidance
To refer for radical radiotherapy or chemoradiotherapy at YGC.
11.6
Morbidity
Post operative complications include:
wound disruption and infection
secondary haemorrhage
femoral nerve damage
hernia
introital scarring and vaginal prolapse
thromboembolic disease
leg oedema
osteitis pubis
urinary infection
sexual function and body image
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Radiotherapy may be the treatment of choice where the patient is unfit for surgery. There is no place for
chemotherapy for the management of vulval carcinoma.
11.7
Surgical management of non squamous vulval cancer
Carcinomas of the Bartholin’s gland are rare vulval cancers and histologically may be of a wide range
of types. The current evidence base is insufficient to suggest different management from squamous
tumours.
Basal cell carcinomas and verrucous carcinoma are rarely if ever associated with lymph node
metastases and need wide local excision only. Basal cell carcinomas require a 1cm margin of excision.
Basal cell carcinoma can also be treated with radiotherapy, which should be the preferred treatment
modality if resection would compromise function.
Malignant melanomas have not been shown to benefit from block dissection of the groin. Wide local
excision is preferred as relapse rates in this group are high and correlate closely to the depth of invasion
(Clarke’s levels or Breslow’s thickness).
12.
Dealing with recurrent disease
Twenty six per cent of patients develop recurrent disease (Podratz et al, 1982) and is usually on the
vulva. Vulval recurrences are best treated with further surgical excision ensuring adequate clear
margins of resection. Groin recurrence should be treated with radiotherapy if radiotherapy has not been
offered before (grade C recommendation).
All cases must be discussed at the gynae cancer MDT to decide further treatment.
13.
13.1


Treatment algorithms for patients with carcinoma of the vulva
Primary therapy
Patients with operable primary tumours and negative nodes clinically and on imaging –
primary surgery to tumour and ipsi/contralateral nodes depending on size and location.
Patients with operable primary tumours but positive nodes clinically or on imaging – confirm
nodal status by FNA.
If only one small involved node on imaging or FNA – surgery for primary tumour and
lymph node dissection on one or both sides depending on laterality of primary.
If more than one node involved - surgery to primary tumour. Lymph node dissection
to one or both sides depending on the laterality of primary or external radiotherapy to
inguinal and lower pelvic nodes with concomitant chemo + electron boost to involved
nodes.
If node is fixed – surgery to primary tumour and external radiotherapy to inguinal and
lower pelvic nodes with concomitant chemo + electron boost to involved nodes.

Include primary site and ‘skin bridge’ in phase I external radiotherapy to pelvic and
inguinal nodes. These tissues need not be included in the phase II electron boost.
Patients who have inoperable primary tumours.
No nodes clinically or on imaging – external radiotherapy to tumour with
concomitant chemo. Consider surgery for residual primary disease or electron or
brachytherapy boost to primary.
Positive nodes clinically or on imaging – confirm by FNA if doubtful, external
radiotherapy to primary tumour, inguinal and lower pelvic nodes with concomitant
chemo, electron or brachytherapy boost to primary and electron boost to involved
nodes.
13.2


Adjuvant therapy to the primary if:
Margins of excision <8mm - some margins may have to be <8mm if major morbidity is to be
avoided (for instance the margin from the urethra). Occasionally it may be reasonable to
accept a narrow margin of 4-6mm and then follow the patient closely. Otherwise consider reexcision or external radiotherapy to primary site.
Margins are microscopically or macroscopically positive - consider further surgery or external
radiotherapy to primary site with electrons or consider brachytherapy implant/ mould.
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
13.3
13.4



If nodes are fixed and are to be irradiated - include primary site and ‘skin bridge’ in phase I
external radiotherapy to pelvic and inguinal nodes. These tissues need not be included in the
phase II electron boost to the nodes.
All cases must be discussed at the gynae cancer MDT to decide further treatment.
Adjuvant therapy to the lower pelvic and inguinal nodes if:
Post surgery:
>1 node found on either side or 1 node with >50% tumour replacement or
extracapsular nodal spread. External radiotherapy to the lower pelvic and inguinal
nodes. Do not include primary site unless there are narrow margins or nodes are
fixed.
Individualised therapy for patients with recurrent or metastatic disease
Patients with locally recurrent disease – radical surgery or chemo-radiotherapy as appropriate.
Consider brachytherapy.
Patients with untreatable local disease or metastatic disease – palliative chemotherapy.
Consider:
Cisplatin/ Methotrexate/ Bleomycin q3-4/52
Cisplatin/ Mitomycin C/ Irinotecan q2/52
Single agent Taxol
Gemcitabine
All cases must be discussed at the gynae cancer MDT to decide further treatment.
13.5
Pelvic exenteration
Pelvic exenteration is an option where there is no evidence of extra-pelvic disease and the pelvic
recurrence is central. This usually involves concomitant removal of the bladder or rectum or both.
Careful pre-operative counselling with a multi-specialist team is essential. Five year survival may
approach 50% but survival at 5 years is rare in node positive patients.
Patients are to be referred to Liverpool Womens Hospital if exenteration to be considered and
all cases must be discussed at the gynae cancer MDT to decide further treatment.
13.6
Palliative care (see Palliative care file)
The provision of palliative and supportive care for patients with gynaecological malignancies should be
an integral part of service provision. The NICE guidance Improving Supportive and Palliative Care for
Adults with Cancer was published in March 2004 and provides detailed recommendations which
complement and inform this guidance (NICE, 2004).
There is little robust evidence from the palliative care literature that is specific to patients with
advanced gynaecological malignancies, therefore this guidance is based on evidence from studies
looking at patients with a broad range of advanced malignancies.
14.
Survival
For patients without groin node involvement the 5 year survival is about 90%. For those with groin
node involvement it is around 50% and with pelvic node involvement it is about 20%.
14.1
Cancer dataset/ inventory of active trials
CaNISC data items to be developed.
Active trials – nil.
15.
Follow up
As patients who relapse locally with vulval carcinoma have a good chance of cure and / or prolonged
remission with prompt re-treatment, the patient should be followed up in an environment where trained
personnel are available to recognise the earliest signs or symptoms of recurrence at the cancer centre or
unit.
Follow up should be performed 3 monthly for 2 years, 6 monthly for 1 year and then annually.
All patients must be encouraged to report any symptoms suggestive of recurrent disease immediately by
contacting their CNS rather than wait until their next outpatient appointment.
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15.1
Identification and management of late effects of treatment
Pyschosexual, emotional, bowel, genitourinary, neuropraxia other problems may need detailed
discussion with the clinical nurse specialist and psychological, lymphoedema, pain, spiritual and other
support services.
16.
Contact Names/ Numbers
Simon Leeson
Obstetrician and Gynaecologist YG (t 01248 384954); CNS Sr Liz Hall (t 01248 385003)
Philip Toon
Obstetrician and Gynaecologist NEWT (t 01978 725834)
Nigel Bickerton Obstetrician and Gynaecologist YGC (t 01745 534655)
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17.
Algorithm
Algorithm for primary management of patients with vulval cancer
Stage I/II
Pre op
Biopsy
+ CT scan abdo/pelvis
+ FNA suspicious nodes
Stage Ia
WLE
Stage Ib/II central
triple incision
vulvectomy
Stage Ib/II lateral
WLE/unilateral
pelvic
lymphadenectomy
If FNA +ve >1 node (upstage to
III/IV)
option to forego groin dissection and
add chemorad to pelvis/groins
Stage III/IV
Bulky vulva
chemorad to vulva
+
butterfly/triple incision
vulvectomy*
Fixed nodes
ERT to pelvis and groins
+
simple vulvectomy*
Multiple mobile nodes
vulvectomy/WLE + groin
dissection
or
chemorad to pelvis/groins
Adjuvant external beam radiotherapy if:
> 1 node involved on either side; any node with >50% tumour replacement; extracapsular
spread
* consider skin grafting
chemorad = chemoradiation
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18.
Summary
Pre-op assessment
 vulval biopsy
 cervical smear - if not recently performed and cervix in situ
 Hb, U+E, liver function tests, 2 unit cross match
 chest X-ray
 CT scan abdo/pelvis - for disease clinically involving groin nodes
 FNA – if suspicious groin nodes
Surgery
 Stage Ia disease (up to 1mm depth of invasion or ‘microinvasion’) wide local excision (2cm tumour clear margins).
 Verrucous carcinoma/ Vulval melanoma/ Paget’s disease of the
vulva - wide local excision (2cm clear margins), simple vulvectomy.
 Basal cell carcinoma - wide local excision (1cm margins).
 Disease extending deeper than 1mm invasion - radical vulvectomy.
Bilateral groin dissection is achieved with a triple incision for stage Ib
and II disease and wide local excision (2cm clear margins). Consider
ipsilateral groin dissection only for lateral tumours. Modified butterfly
incision for stage III-IV disease.
 Vulval recurrence - further wide local excision.
Radiotherapy
 if more than 1 positive groin node, >50% nodal tumour replacement,
extracapsular extension or <8mm of resection margin at vulvectomy.
 if unfit for primary surgery.
 for groin recurrence after primary surgery.
Chemoradiation
 fixed bulky vulval disease. Additional surgery decided on a case by
case basis.
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19.
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Classification of evidence levels
Ia
Evidence obtained from meta-analysis of randomised controlled trials
Ib
Evidence obtained from at least one randomised controlled trial
IIa
Evidence obtained from at least one well designed controlled study
without randomisation
IIb
Evidence obtained from at least one other type of well designed quasiexperimental study
III
Evidence obtained from well designed non-experimental descriptive
studies, such as comparative studies, correlation studies and case
studies
IV
Evidence obtained from expert committee reports or opinions and/or
clinical experience of respected authorities
Lower limit of acceptable evidence base is level IIa.
Grades of recommendation
A
Requires at least 1 randomised controlled trial as part of a body of
literature of overall good quality and consistency addressing the
specific recommendation
B
Requires the availability of well controlled clinical studies but no
randomised clinical trials on the topic of recommendation
C
Requires evidence obtained from expert committee reports or opinions
and/or clinical experiences of respected authorities. Indicates an absence
of directly applicable clinical studies of good quality
Lower limit of acceptable grade of recommendation is B.
North Wales Cancer Centre Guideline Group
Simon Leeson
Philip Toon
Nigel Bickerton
Obstetrician and Gynaecologist (chair)
Obstetrician and Gynaecologist
Obstetrician and Gynaecologist
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