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Vulval Cancer Guidelines V3.0

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Vulval Cancer Guidelines V3.0 Gynaecology NSSG (Lancs & South Cumbria) Vulval Cancer Guidelines V3.0 ** VALID ON DATE OF PRINTING ONLY – all guidelines available on the Strategic Clinical Network website : GMLSC SCN Date First Published 1st December 2009 v1.0 Date of last revision 20 June 2014/July 2015 V3.0 Date for next revision 2017 VULVAL CANCER GUIDELINES EPIDEMIOLOG Y Vulval cancer is rare and accounts for approximately 3-5% of all gynaecological malignancies. In 2005 in England and Wales there were 903 cases (Incidence rate of 3.3 per 100,000 women) and 327 deaths (Death rate 1.4 per 100,000 women) Cancer Research UK 2009. The disease occurs in an older age groups between the ages of 60 and 75, however, there is an increasing number of invasive tumours are being found in younger women, especially those who are immuno-compromised. However the incidence has not risen significantly in the last twenty years. Vulval maturation disorders e.g. lichen sclerosus, and Vulval Intraepithelial Neoplasia (VIN) are known to predispose to vulval cancer. Lichen sclerosus mainly affects older women and has a 3-5% progression rate to invasive disease(1). Differentiated VIN tends to be associated with Lichen Sclerosus and is not HPV associated. About 70-90% of classical or Bowenoid VIN contain HPV DNA while the detection rate of HPV DNA in invasive vulval SCC is 20–60%. HPV is most strongly linked with tumours in younger women, with an 11-fold risk increase reported for vulval intraepithelial neoplasia (VIN) and early- stage cancer in women under the age of 45 with serological evidence of HPV infection, but no increase in women over this age. An increasing number of younger women are presenting with HPV- related VIN. Other rare conditions that pre-dispose to vulval cancer are Paget’s disease of the vulva and vulval melanoma in situ. PATHOLOGY Squamous cell carcinoma Melanoma Bartholin gland tumours Adenocarcinoma Basal cell carcinoma Pagets disease 2 FIGO STAGING OF VULV A L C A N C E R FIGO (2009) staging and TNM comparison Stage (FIGO) Description T N M Primary tumour cannot be assessed TX No evidence of primary tumour T0 Stage 0 Carcinoma in situ (preinvasive carcinoma) Tis Stage I Tumour confined to vulva and perineum. No T1 nodal metastasis. IA Tumour confined to vulva or perineum, ≤ 2 cm in T1a greatest dimension and with stromal invasion ≤ 1.0 mm. No nodal metastasis. IB Tumour confined to vulva or perineum, > 2 cm in T1b greatest dimension and with stromal invasion > 1.0 mm. No nodal metastasis. Stage II Tumour of any size with extension to adjacent T2 perineal structures (lower 1/3 urethra; lower 1/3 vagina; anus). No nodal metastasis. Stage III Tumour of any size with or without extension T1.T2 N1 M0 to adjacent perineal structures (lower 1/3 T3 N0.N1 M0 urethra; lower 1/3 vagina; anus) and with positive inguinal-femoral nodal metastasis. IIIa (i) With 1 lymph node metastasis ≥5mm or (ii) 1-2 lymph node metastasis <5mm IIIb (i) With 2 or more lymph node metastasis ≥5mm or (ii) 3 or more lymph node metastasis <5mm IIIc With lymph node metastasis with extra-capsular spread Stage IV Tumour invades other regional (upper 2/3 urethra, 2/3 vagina) or distant structures Stage IVA Tumour invades any of the following: T1.T2. N2 M0 (i) Upper urethral and/or vaginal mucosa; T3 bladder mucosa, rectal mucosa; or T4 any M0 fixed to bone or (ii) Fixed or ulcerated inguinal-femoral lymph nodes. Stage IVB Any distant metastasis including pelvic lymph nodes any any M1 The depth of invasion is defined as the measurement of the tumour from the epithelial-stromal junction of the adjacent most superficial dermal papilla, to the deepest point of invasion. PRESENTATION AND DIA GNOSIS A suspicion of vulval cancer should be raised by vulval ulceration, vulval lump or non resolving vulval irritation or discomfort. Vulval warts are uncommon in elderly women and should be viewed with suspicion. These patients should be seen and managed by the Cancer leads in the network hospitals. 3 Diagnosis is based upon a representative biopsy of the tumour that should include the area of epithelium where there is a transition of normal to malignant tissue. These can generally be obtained with local anaesthetic as an out-patient. Diagnostic biopsies should be of a sufficient size (greater than 3 mm) to allow measurement of depth of invasion and orientated to allow quality pathological interpretation. In general wedge/punch on small tumours will suffice. For small suspicious lesions, women should be referred to the gynaecological cancer centre, either after a small biopsy that leaves the lesion identifiable or no biopsy at all. The site and size of the lesion are important variables in treatment planning and these should be assessable at the centre. Careful examination of the lesion is mandatory and appropriate documentation of the size and location is important. Suspected spread to adjacent structures (e.g. urethra, anus, bone) should be noted. Both groins should be examined. DO NOT PERFORM WIDE LOCAL EXCISION IF INVASIVE DISEASE IS SUSPECTED. Excision biopsies prior to referral should be avoided as these are usually insufficient as treatment and may compromise definitive surgery. If sentinel node identification is to be performed accurately, the radio-isotope dye and methylene blue dye need to be injected at the tumour site and prior WLE of the whole lesion compromises this technique. Ideally, consideration should be given to obtaining photographic representation of all lesions, if possible. Referral should include sending all relevant histopathological material to the specialist gynaecological pathologist team in the gynaecological cancer centre. All new cases of vulval cancer should be discussed at the cancer centre multidisciplinary team meeting. PRE - TREATMENT INVESTIGAT ION Staging is surgico-pathological. Imaging is not routinely indicated if disease is less than 2cms in diameter and no palpable lymph nodes. MRI In patients with locally advanced disease, MRI should be performed to assess extent of disease and identify inguinal and pelvic lymph node metastases. In Lancashire and South Cumbria Network, we have elected to image by MRI all disease ≥Stage 1b to asses nodal status and local invasion. CT CT is an alternative if radiotherapy is the likely treatment option. EUA Where locally advanced disease is suspected or where there is doubt about the resectability of the tumour, examination under general anaesthesia (EUA) may be required in order to plan further management. EUA may be required in order to obtain a diagnostic biopsy in a woman who is very symptomatic with pain. 4 Consideration should be given to performing a joint EUA where necessary, involving other relevant team members such as plastic/colorectal surgeons or a clinical oncologist. This should be arranged and carried out by the centre clinician. If there are clinically suspicious nodes patients should undergo imaging as described above. There is then the option of proceeding with resection of the primary lesion and formal inguino-femoral lymphadenectomy (IFL) or resection of the primary lesion and debulking of the lymph node with a view to post-operative irradiation of the groin(s). Lymphadenopathy determined by imaging should use a threshold of short axis dimension of ≥15mm or radiological concern of appearance. There is an option of either proceeding with IFL or sampling using fine needle aspiration (FNA) or trucut biopsy. This should be done under ultrasound control and is done by a radiologist. If the FNA/biopsy is negative then proceed to IFL or if positive then consider the option of radiotherapy to the groins as an alternative to IFL in patients not suitable for surgery (Van der Velden et al, Cochrane Review 2011). HIV TESTING Patient’s diagnosed with Vulval cancer should be counselled about the possible association with HIV and offered testing; this can be done in the Gynaecology clinic if appropriate counselling is available, or by referral to GUM. Human immunodeficiency virus (HIV) is classified by the International Agency for Research on Cancer (IARC) as a probable cause of vulval cancer, based on limited evidence. Vulval/vaginal cancer risk is 6.5 times higher in people with HIV/AIDS compared with the general population, a meta-analysis showed. This is probably due to co-infection with, and reduced capacity to clear, Human papillomavirus (HPV). Vulval/vaginal cancer risk among people with HIV does not vary by level of immunosuppression, a cohort study showed. Vulval/vaginal cancer risk among people with HIV has increased over time (probably related to changing HPV prevalence), a cohort study showed Information taken from Cancer research UK TREATMENT SURGICAL TREATMENT Surgery is the standard treatment of vulval cancer and less mutilating surgery is now employed; For small tumours of < 2 cms and < 1 mm invasion, excision with the intention of a 15 mm (unfixed/fresh) surgical margin is sufficient. Excision Margins Adequate disease free margins are important as these are associated with risk of recurrence and disease free survival. The risk of recurrence increases as the disease free histological margins decrease (> 8.0 mm: 0%; 8.0-4.8 mm: 8%; < 4.8 mm: 54%)(2). In a large published audit of Vulval Cancer management in the South West of England 33% (44/134) had margins less than 8 mm(3) and in the Netherlands this approached 50%(4). In anterior lesions encroaching the urethra, some surgeons might elect to preserve urethral function, if excision of the distal urethra is unlikely to completely excise disease with the knowledge adjuvant radiotherapy will be given. In a single publication from the UK, a suggested standard would be that >90% of cases should have excision margins >3mm(5).
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