Gynaecology NSSG (Lancs & South Cumbria) Vulval Cancer Guidelines V3.0

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1 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 1 st December 2009 v1.0 Date of last revision 20 June 2014/July 2015 V3.0 Date for next revision 2017

2 VULVAL CANCER GUIDELINES E P I D E M I O L O G 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 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 earlystage 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 HPVrelated VIN. Other rare conditions that pre-dispose to vulval cancer are Paget s disease of the vulva and vulval melanoma in situ. P A T H O L O G Y Squamous cell carcinoma Melanoma Bartholin gland tumours Adenocarcinoma Basal cell carcinoma Pagets disease 2

3 FIGO (2009) staging and TNM comparison F I G O S T A G I N G O F V U L V A L C A N C E R 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 urethra; lower 1/3 vagina; anus) and with T3 N0.N1 M0 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; bladder mucosa, rectal mucosa; or T3 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. P R E S E N T A T I O N A N D D I A G N O S I S 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

4 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. P R E - T R E A T M E N T I N V E S T I G A T 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

5 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 SURGICAL TREATMENT T R E A T M E N T 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%; 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). 5

6 The disease free margin is the measured histological margin on the fixed specimen; hence it is inevitably less than the intra-operative surgical margin. It is therefore essential to aim for an intraoperative margin of at least mm on the fresh surgical specimen. Radiotherapy is indicated in squamous disease if excision margins are less than 5 mm from invasive disease. Cases of vulval melanoma and Paget s disease will require larger surgical margins of at least 2 cm. Radical treatment should not be undertaken without prior biopsy confirmation of malignancy. Ilio-femoral lymphadenectomy. Superficial inguinal node dissection alone is associated with increased recurrence rates when compared to combined superficial and deep groin node dissection(6). Therefore a full deep and superficial groin node dissection should be performed. Only 2-3 deep nodes are present and these are located medial to the femoral vein(7). Removal of the deep fascia lateral to the femoral vein can be argued to be unnecessary as there are no nodes lateral to the femoral vein. There are no superficial nodes adjacent to the anterior iliac spine and therefore the superficial circumflex vessels should represent the lateral landmark of dissection(7). In a large Gynecological Oncological Group (GOG) prospective study in the US of 113 women who underwent superficial lympadenctomy only there was no correlation with recurrence rates and the number of nodes removed(8). Standard UK practice is to perform an en-bloc dissection. Recent evidence has suggested that sparing the saphenous vein reduces short and long term morbidity without compromising the number of nodes removed(9). In a single and therefore potentially biased paper outlining standards for surgery, a suggested standard should be that a minimum of 4 nodes should be removed at ilio-inguinal lymphadenectomy(5). Pelvic node dissection should not be performed. Sentinel lymph node dissection Sentinel groin node detection is suitable for tumours up to 4cm in diameter; above that the reliability of the technique is poor. If the node(s) is/are negative, no further treatment will be offered, and the patient will commence follow up. If positive, then adjuvant treatment according to this guideline will be offered. This technique has the potential to reduce the surgical morbidity to the groins and lower limbs in patients with early vulval cancer (stages I and II), the majority of whom (70%) will have negative groin nodes. The negative predictive value of a negative sentinel groin node in vulval cancer has been reported to approach 100%. The GROINSS-V study reported on the safety of performing sentinel groin node dissection in early stage I and II vulval cancers, of less than 4 cm. Groin recurrence rate was low at 2.3%. This compares favourably with patients undergoing a formal inguinofemoral lymphadenectomy. It is notable however that at least 50% of groin recurrences in the GROINSS-V study occurred in patients with multi-focal disease and where there were protocol violations. 6

7 Stage IA disease (<1 mm DOI) Wide local excision, observing the above margins is sufficient. Inguino-femoral lymphadenectomy should not be performed as the risk of nodal metastasis is very low (< 3%) and the associated morbidity of lymphadenectomy is high. All cases must be discussed at the Central MDT and definitive surgery may be done centrally to comply with IOG. Stage 1B disease Where invasion exceeds 1mm, Radical Wide Local Excision of the primary lesion is required, with macroscopic surgical excision margins of 15mm as above. Inguino-femoral Sentinel Node detection should be undertaken as above. Lateralised lesions: these are defined as the leading medial tumour edge being at least 10 mm away from the midline. These are treated with radical wide local excision (with adequate margins as above) in addition to unilateral inguinofemoral lymphadenectomy. Women undergoing ipsilateral dissection and lymphadenectomy should have clinically negative nodes, less than 5 mm DOI, no significant LVSI. If the ipsilateral groin nodes contain metastasis, then the contralateral groin nodes need removal. Midline lesions: with midline lesions, deeper lesions (> 5 mm) or where there is significant LVSI, or when clinical groin adenopathy is present, management consists of radical local excision and bilateral inguinofemoral node dissection. Stage II disease Standard treatment is radical wide local excision or radical vulvectomy (depending on the site and size of the tumour) and bilateral inguinofemoral lymph node dissection The use of a triple incision technique (separate incisions for the groins) has significantly reduced the surgical morbidity associated with this procedure. Unless required by virtue of the size of the lesion, the radical vulvectomy should be modified to a triple incision procedure. If it is considered that adequate excision will leave too large a defect, and a rotational flap is required, then the procedure should be undertaken with a plastic surgeon. An alternative to consider, especially when sphincters are involved, is primary chemo-radiotherapy. A joint EUA between the gynaecology oncologist and oncologist will be required to make these decisions. Reconstructive surgery should be made available to all women where appropriate. This may require plastic surgery input. Vulval reconstruction may help reduce the long term psychological impact for this group of women and may also improve the long term vulval appearance and function. Detailed discussion with a clinical nurse specialist or counsellor specialising in sexual issues is valuable in these circumstances. Plastic Surgery Skin Flaps Ideally patients being considered for plastic surgery procedures should have a consultation with the plastic surgeon pre-operatively to discuss the proposed procedure and any associated risks. Where primary radical surgery is expected to compromise sexual function, psychosexual counselling should be offered prior to any joint plastic reconstructive procedures. 7

8 Surgical Complications Radical WLE or vulvectomy is associated with the following complications, and patients should be councelled accordingly. Introital stenosis Posterior vaginal / perineal prolapse Perineal pain Faecal incontince Femoral hernia Pubic osteomyelitis Fistulae Dyspareunia. Urinary incontinence Hooding of the urethra Dyspareunia Psychosexual morbidity. Inguino-femoral lymphadenectomy can lead to: Parasthesia Lymphocyst Lymphoedema. Advanced, Stage III and IV, Vulval Cancer Approximately one third of patients with vulval cancer present with stage III and IV disease and are characterised by local extension resulting in serious complex problems. Positive groin nodes are found in more than 50% of these patients and are often ulcerating and/or fixed to the femoral vessels. Therefore, in many patients, the standard radical surgery may not be enough and either tailored ultraradical/exentrative surgery or non-surgical treatment should be considered. Many of these women are elderly and treatment plans should be individualized taking into account performance status and morbidity of treatment. The following are required as a minimum: Formal assessment of co-morbidity, systems function and performance status with the relevant blood and functional tests. Assessment of the extent of the disease with pelvic MRI scan. Abdominal and chest CT are only required where advanced disease is expected. Joint EUA involving, the Gynaecological oncologist, clinical oncologist and plastic, colorectal or urology surgeons depending on the extent of disease and involved structures. Early involvement of the palliative care team. Discussion of the care plan and prognosis with the patient and/or carers to ensure that the treatment plan is based on the individual needs of that patient. Tumour deemed suitable for primary resection Radical excision is appropriate and may be in the form of radical vulvectomy, modified radical vulvectomy as a joint procedure including plastic reconstruction, as determined by the extent of tumour. Bilateral groin node dissection or debulking of suspicious enlarged/ulcerating groin nodes is also performed. This should be tailored to each individual case. Adjuvant pelvic and groin irradiation is given if one or more groin nodes are involved. There is no role for pelvic lymphadenectomy. 8

9 Primary Chemo-radiotherapy Primary (chemo)radiotherapy is indicated for women deemed unsuitable for ultra-radical surgery and may allow subsequent sphincter-preserving surgery. Good disease control can be achieved. Surgery may be performed following completion of treatment to remove residual disease. In cases of fixed or ulcerating groin nodes, debulking of groin nodes may be considered prior to radiation treatment if feasible. Alternatively radiotherapy should be considered. There is insufficient evidence to suggest the superiority of one treatment over the other. There is no consensus as to whether to perform groin dissection after primary radiotherapy treatment where there has been a complete response. Surgery in an irradiated groin is associated with significant morbidity. Adjuvant radiotherapy The need for adjuvant radiotherapy is based upon the groin node status and the surgical margins. There is not enough evidence for routine radiotherapy to the vulva in patients with close but negative margins. Where the closest pathological margin is <5mm consideration should be given to further local excision, although evidence is lacking that this will result in a reduction in local recurrence. If further surgery is not feasible, then adjuvant radiotherapy should be considered if the margins are less than 5mms. Adjuvant radiotherapy to the groin(s) is administered if either groin has one or more nodes involved with microscopic metastatic disease.. If an unilateral groin node dissection has been performed and there are positive nodes then consideration should be considered for contralateral node dissection or contralateral groin radiotherapy. LYMPHOEDEMA In gynaecological cancer, swelling of one or both legs in the absence of hypoalbuminaemia or vein thrombosis is usually due to lymphatic obstruction. This may be a consequence of treatment itself or active pelvic disease. The cancer centre team or the team in the Units have the responsibility to refer such patients to a specialist lymphoedema service for assessment and management. Proactive treatment can significantly reduce lymphoedema and control swelling even in the presence of progressive disease. Acute infective episodes may present a florid cellulitis but frequently may be a case of mild erythema and general malaise. These should always be actively treated with Penicillin V 500 mgs q.ds.x 2 weeks (alternatively Erythromycin in those with sensitivity to Penicillin). Severe episodes may require inpatient treatment. UNUSUAL TUMOUR MORPHOLOGY Malignant melanoma S P E C I A L C I R C U M S T A N C E S Malignant melanoma is the second commonest malignant vulval tumour. Management should be individualized and cases should be discussed in gynae and melanoma MDT. Treatment is by radical local excision with surgical margins of at least 2cm. In the absence of enlarged nodes, groin node dissection is not indicated. 9

10 Sentinel node detection has been found to be useful for skin melanomas of depth 1-4mm, but NOT for mucosal melanomas. BRAF testing should be done to inform the choice of oncology treatments. Lancet Oncol Feb;14(2):e60-9. doi: /S (12) Which drug, and when, for patients with BRAF-mutant melanoma? Patients with metastatic melanoma had few treatment options until 2011, when two drugs-ipilimumab and vemurafenib-were approved following advances in the understanding of melanoma biology and tumour immunology. Almost 50% of melanomas harbour mutations in BRAF, mainly at codon 600, which result in constitutive activation of the MAPK pathway. The selective inhibitors of mutant BRAF Val600, vemurafenib and dabrafenib, showed major tumour responses, resulting in improved progression-free and overall survival in patients with metastatic disease, compared with chemotherapy. Antitumour activity was also recorded in brain metastases. The growth of cutaneous squamous-cell carcinomas is a unique side-effect of BRAF inhibitor therapy that is induced by the paradoxical activation of the MAPK pathway in cells with RAS mutations CT of chest, abdomen and pelvis are performed to exclude widely metastatic disease. The most useful prognostic indicator for vulval melanoma is Breslow s thickness. In the absence of obvious metastases, careful clinical follow-up is usually advised. Breslow s Depth of Invasion (thickness) Breslow s DOI is a measurement of the depth of the lesion measured vertically in mm from the top of the granular layer (or base of superficial ulceration) to the deepest point of tumour involvement. Tumours are classified according to the depth: < or equal to 0.75mm mm mm > or equal to 4mm Bartholin gland carcinoma This is a rare form of vulval cancer that tends to be deeply seated and associated with metastatic disease. It is managed in the same way as squamous carcinoma of the vulva. The close proximity to the anal canal may necessitate partial resection and temporary colostomy. Basal cell carcinoma and Verrucous carcinoma These squamous variants are rarely associated with lymph node metastasis and can be managed by wide local/radical excision. Groin node dissection is not indicated. In basal cell carcinoma, radiotherapy treatment should be considered if surgical resection is thought to compromise sphincter function. Groin dissection should also be omitted in cases of verrucous tumours of the vulva, basal cell carcinoma and malignant melanoma (as above) 10

11 F O L L O W U P Routine follow up of patients treated for vulval cancer serves two purposes. It may provide the opportunity to detect asymptomatic recurrent disease but, as importantly, will allow the identification and management of treatment related morbidity, psychosexual and psychosocial issues. The schedule and nature of follow up should be determined for each individual as defined in the L&SCCN Gynaecology NSSG Follow up Guidelines. Follow up should be at the local hospital and consist of a clinical history and examination. If symptoms or signs suggest possible recurrence, appropriate investigations, such as a CT scan to evaluate metastatic disease, should be requested. Patients with evidence of recurrence should be discussed at the Specialist MDT. For patients who have remaining VIN (e.g. positive margins for VIN) the use of vulvoscopy should be considered. P S Y C H O S E X U A L S E Q U A L A E Genital tract cancer inherently involves psychosexual issues (Barton, 2003, p.689), the trauma of which has be managed with the actual diagnosis itself. Quality of life and of sexual life has to be a key part of all care outcomes (Graziottin, 2003, p.129). A 2003 study (Ekwall, Ternestedt & Sorbe) of gynaecological cancer patients found that sexuality was one of the main three issues of central importance to maintain this quality. Survivorship is now a huge issue as treatments for cancer improve, services have to be in place to improve support for these patients (Department of Health, 2007, p.14). Treatments, though often curative are associated with considerable morbidity including vulval oedema, vaginal discharge, lymphoedema, lymphocysts, wound infections and immobility. Psychosexual issues include loss of femininity due to changes in body images and disfigurement, loss of sexual desire, depression, loss of confidence and self esteem (Allen, 2003, p.599), some of which are underlying pre diagnosis and are not therefore true treatment sequalae, but nevertheless can have a huge impact on morbidity. Green, Naumann, Elliott, et al (2000) found that sexual dysfunction was not specifically due to the type of surgery, nodal dissection or amount of excised tissue, this in direct contrast to Thuesen, Andreasson & Bock (1992) who found less sexual disruption in women who had local excision compared to more radical surgery. This possibly suggests that dysfunction is very individual and may relate to the intimacy and relationship aspect rather than the disfigurement. What all parties agree on is more research on this aspect which at present is seriously lacking. Although normally a disease of the elderly population, 10% of patients are now younger than 40 years of age (Barton, 2003, p.683), which further emphasises the need to prevent sequalae with treatment planning and counselling before this commences if possible. Health professionals should not assume that the elderly patient is no longer sexually active just because of age, all patients should be assessed on an individual basis, although it is acknowledged that many women may not readily volunteer information on this subject (Allen, 2003, p.596). Pre treatment assessment in all patients can yield valuable information, routine discussions on sexual history with the patient and her partner can normalise the subject of sexuality and highlight potential concerns which can be addressed (Quinn, 2007, p.286). Improving Outcomes in Gynaecological Cancer guidelines (1999, p.22) advise that all sexually active patients be offered specific information 11

12 on the effects of treatment on their relationship and be advised of the specialist role of the CNS. The advanced communication skills, expertise and confidence in discussing sexuality issues which are encompassed within the CNS role suggest this route is the most advantageous (Allen, 2003, p.596). Tools utilised to enable a thorough assessment include the PLISSIT model (Parkinson & Pratt, 2005), which alerts the patient that a discussion on sexual issues is permissible and limited information involves providing data on the discussed sexual problem. Specific suggestions details discussions about treatment interventions and options which may help with the stated problem and intensive therapy normally implies referral to a specialist. The PLISSET model can be used pre or post treatment to further investigate a psychosexual problem or dysfunction, the distress thermometer, a more recent innovation advocated by NICE in regard to guidelines on supportive and palliative care (2004) can be used by health professionals to screen for psychological distress. The distress thermometer is a single item patient assessment checklist scale, short and easy to complete, making it more acceptable to the patient. Previous tools such as the Hospital Anxiety and Depression Scale (HADS) has proved cumbersome in certain situations with low sensitivity and specificity. The distress thermometer boasts sensitivity of 97%, specificity of 67% (Marie Curie Research Unit, Royal Free & UCLH, 2006) on a scoring range of 1-6, cut off at 4 to achieve the high sensitivity which concurs with previous international studies. These models can simplistically guide the health professional through evaluation of sexual dysfunction whilst providing relevant information. The results of which could yield simple interventions such as the use of vaginal lubricants and altered sexual positions for significant impact results. A 2001 study (Maughan & Clark, 2001) piloted the CNS as a pre and post operative psychosexual counsellor, which found sexual function much improved compared to no counselling and twelve week psychological group therapy sessions also enhanced immediate sexual function though long term benefits are unknown (Caldwell et al, 2003). If the pre and post counselling sessions can utilise one of the aforementioned tools and adequate time allocated to accomplish this, some aspects of psychosexual sequalae maybe reduced. R E C U R R E N T D I S E A S E Both treatment and prognosis depend upon the site and extent of recurrence. Treatment plans should be made within the context of a multi-disciplinary team. Thorough preoperative assessment, including CT of chest, abdomen and pelvis, is of paramount importance in defining the objective of treatment (curative or palliative) and optimizing the treatment outcome. Radical excision of localized recurrence gives an approximate 5-year survival rate of 56% when the regional nodes are not involved. In radiation naive patients radiotherapy should be considered if surgery is likely to impair function. An exenterative procedure may be an option in individual patients. Groin node dissection, unilateral or bilateral, may be considered if not done previously. Indications for post-operative radiotherapy following excision of local recurrence are not clear. Groin recurrence carries the worst prognosis and treatment options are often limited. In those who have not received groin irradiation, radiotherapy with or without additional surgery should be considered. If the groins have already been irradiated, palliation (either surgical or systemic treatment) should be considered as early as possible once recurrence is established. Systemic chemotherapy after relapse is used depending upon the patients general condition and performance status as well as previous response to any chemotherapy regimes. 12

13 Patients considered to within one year of dying should be referred to Palliative Care see End of Life Strategy Group. A R E A S O F R E S E A R C H A N D D E V E L O P M E N T The L&SCCN Gynaecology NSSG and Gynaecology Specialist MDT keep an up to date record of clinical trials open to recruitment on the NCRI research portfolio. A quarterly report is presented to the L&SCCN Gynaecology NSSG on research activity available for gynaecological cancers. 13

14 R E F E R E N C E S 1. Maclean AB, Jones RW, Scurry J, Neill S. Vulvar cancer and the need for awareness of precursor lesions. J Low Genit Tract Dis Apr;13(2): Heaps JM, Fu YS, Montz FJ, Hacker NF, Berek JS. Surgical-pathologic variables predictive of local recurrence in squamous cell carcinoma of the vulva. Gynecol Oncol Sep;38(3): Falconer AD, Hirschowitz L, Weeks J, Murdoch J. The impact of improving outcomes guidance on surgical management of vulval squamous cell cancer in southwest England ( ). BJOG Apr;114(4): De Hullu JA, Hollema H, Lolkema S, Boezen M, Boonstra H, Burger MP, et al. Vulvar carcinoma. The price of less radical surgery. Cancer Dec 1;95(11): D Arcy TJ, Roy A, Thomas A, McIndoe A, Soutter WP. Standards for the management of cervical and vulval carcinoma. BJOG Jul;107(7): Stehman FB, Bundy BN, Thomas G, Varia M, Okagaki T, Roberts J, et al. Groin dissection versus groin radiation in carcinoma of the vulva: a Gynecologic Oncology Group study. Int J Radiat Oncol Biol Phys. 1992;24(2): Micheletti L, Borgno G, Barbero M, Preti M, Cavanna L, Nicolaci P, et al. Deep femoral lymphadenectomy with preservation of the fascia lata. Preliminary report on 42 invasive vulvar carcinomas. J Reprod Med Dec;35(12): Stehman FB, Ali S, DiSaia PJ. Node count and groin recurrence in early vulvar cancer: A Gynecologic Oncology Group study. Gynecol Oncol Apr;113(1): Dardarian TS, Gray HJ, Morgan MA, Rubin SC, Randall TC. Saphenous vein sparing during inguinal lymphadenectomy to reduce morbidity in patients with vulvar carcinoma. Gynecol Oncol Apr;101(1): van Seters M, van Beurden M, ten Kate FJ, Beckmann I, Ewing PC, Eijkemans MJ, et al. Treatment of vulvar intraepithelial neoplasia with topical imiquimod. N Engl J Med Apr 3;358(14): Tristram A, Fiander A. Clinical responses to Cidofovir applied topically to women with high grade vulval intraepithelial neoplasia. Gynecol Oncol Dec;99(3): Allen, J. (2003) Role of the Clinical Nurse Specialist in Vulval Cancer, Best Practice and Research Clinical Obstetrics & Gynaecology, 17 (4), pp Barton, D. (2003) The Prevention and Management of Treatment Related Morbidity in Vulval Cancer, Best Practice and Research Clinical Obstetrics & Gynaecology, 17 (4), pp Caldwell, R., Classen, C. Lagana, L. (2003) Changes in Sexual Functioning and Mood amongst Women Treated For Gynaecological Cancer Who Receive Group Therapy: A Pilot Study, Journal of Clinical Psychology, 10 pp

15 15. Ekwall, E., Ternestedt, B. Sorbe, B. (2003) Important Aspects of Health Care for Women with Gynaecologic Cancer, Oncology Nurses Forum, 30 pp Graziottin, A. (2003) Oncologic Gynaecologic Surgery and Sexuality, Journal of Psychosomatic Research, 55 p Great Britain. Department of Health (2007) Cancer Reform Strategy. Lon don: DOH. 18. Green, M., Naumann, R., Elliott, M., Hall, J., Higgins, R. and Grigsby, J. (2000) Sexual Dysfunction following Vulvectomy, Gynaecology Oncologic, 77, pp Low, J., Gessler, S., Daniells, E., Williams, R., Brough, V., Toolman, A and Jones, L. (2006) Validating the Distress Themometre as a tool to screen for psychological distress in the UK. Marie Curie Research Unit, Royal Free & UCLH. A19F-B90F-6BEB13C1D03D.htm#distress 20. Maughan, K. and Clarke, C. (2001) The Effect of the Clinical Nurse Specialist in Gynaecology Oncology on Quality Of Life and Sexuality, Journal of Clinical Nursing, 10, pp National Health Service Executive (1999) Guidance on Commissioning Cancer Services: Improving Outcomes in Gynaecological Cancers The Manual. London: NHS. 22. National Institute of Health and Clinical Excellence (2004) Improving Supportive and Palliative Care for Adults with Cancer. London: NICE 22. Parkinson, N. and Pratt, H. (2005) Clinical Nurse Specialists and the Psychosexual Needs of the Patients with Gynaecological Cancer, Journal of British Menopause Society, 11 pp Quinn, M. (2007) Sexual Function after Treatment of Gynaecological Cancer, Sexologies, 16 pp Thuesen, B., Andreasson, B. & Bock, J. (1992) Sexual Function after local excision of VIN, Obstetrics and Gynaecology Scandanavia, 71, pp van der Velden J, Fons G, Lawrie TA. Primary groin irradiation versus primary groin surgery for early vulvar cancer. Cochrane Database of Systematic Reviews 2011, Issue 5. Art. No.: CD DOI: / CD pub2. 15

16 End of Life Pathway The WHO describes palliative care as 'the active, holistic care of patients with advanced, progressive illness'. 1 The hub of any patients medical health is the GP, they are in an ideal position to provide and coordinate this care for a number of reasons: they have long-established relationships with their patients which are so important at this critical time in a patient's life they are used to dealing with co-morbidity and uncertainty they are trained to treat patients holistically which is central to the palliative care approach. GPs have to be able to provide high quality, equitable care, and to work together effectively with specialist teams if they are to provide the best primary palliative care for all who require it. There is an increasing imperative to be able to recognise the needs of all patients nearing the end of their lives, not just those with cancer, and to be able to extend some of the developments in care provided for cancer patients to those with other illnesses, which constitute 75% of all deaths. A large proportion of patients receive news of palliative disease which will lead to end of life from the secondary care and steps need to be put in place to ensure provisions are met, this becomes more relevant when the time frame for commencing end of life provision is approximately one year before death ie at the time of advancing disease. Proactive end of life care In order to provide optimal care for any patient nearing the end of their life, i.e. not just in the terminal or dying phase, but in their last year, we need to be able to do three things: identify where a patient is on their illness trajectory do they have years, months, weeks or days to live? This then allows proactive management, calmer planning and less 'fire-fighting' crisis management assess their needs, and those of their family/carers, in the light of their advance care plan plan (using a management plan) and then provide their care according to the patient's preferences and varying needs, at different times. A key point is for all hospital and hospice clinicians who recognise that a patient may be in their last year of life to notify the patient's GP and recommend that the patient is added to the palliative care register. The basis for this lies in the End of Life Care Strategy. End of Life Care Strategy The strategy was developed over a period of a year by an advisory board led by Professor Mike Richards and six working groups, consulting over 300 stakeholders. It became apparent that a whole systems approach was required. Accordingly the Strategy strongly recommends that a care pathway approach should be followed both for care and the commissioning of end of life care. 16

17 Key Steps Identification of people approaching the end of life, and initiating discussions about preferences for end of life care; Care planning: assessing needs and preferences, agreeing a care plan to reflect these and reviewing these regularly; Coordination of care; Delivery of high quality services in all locations; Management of the last days of life; Care after death; and Support for carers, both during a person s illness and after their death. 17

18 The story of a patient s health from diagnosis of a life-limiting illness can be seen with this model. The model comprises five phases as described below with some examples of practice highlighted. 1. Advancing disease timeframe: 1 year or more. Example of practice required -the person is placed on a supportive care register in General Practitioner (GP) practice/care home. The person is discussed at monthly multidisciplinary practice/care home meetings. 2. Increasing decline timeframe: 6 months [approximate]. Example of practice required -DS1500 eligibility review of benefits, Preferred Priorities for Care (PPC) noted, Advance Care Plan (ACP) in place and trigger for continuing healthcare funding assessment 3. Last days of life timeframe: last few days. Examples of practice required - primary care team/care home inform community and out of hours services about the person who should be seen by a doctor. End of life drugs prescribed and obtained, and Liverpool Care Pathway (LCP) implemented. 4. First days after death timeframe: first few days. Examples of practice required include prompt verification and certification of death, relatives being given information on what to do after a death (including D49 leaflet), how to register the death and how to contact funeral directors 5. Bereavement timeframe: 1 year or more. Examples of practice required include access to appropriate support and bereavement services if required. As health professionals working within gynae oncology, we treat patients who fit all parameters of the end of life scale, what is required of us is to be aware of whereabouts on this scale our patients fit and advise the GP, District Nurses, Macmillan Nurses accordingly so they can be transferred as appropriate to the primary care end of life register so all their needs can be anticipated and met at the primary level. 18

19 References 1. Cancer pain relief and palliative care. Report of a WHO Expert Committee. World Health Organ Tech Rep Ser 1990; 804: egy.asp 3. odel.php NICE 19