Introduction
Cancer of the vulva cancer is a rare gynaecological malignancy and according to Cancer Research UK, 1 there were around 1,300 new cases of vulval cancer in the United Kingdom in 2014, giving in the region of approximately four new cases a day. It accounts for less than 1% of all new cases in the United Kingdom and is the 20th most common female cancer. 1 It is a disease commonly diagnosed in older women, however, vulval cancer incidence rates have increased overall for the younger adult age groups, in England, since the mid-1970s. The rise in younger women may be linked to the increase in human papillomavirus infection, the overall incidence of vulval cancer has increased as a result of more new diagnoses in the under 70-year age group.Reference Lai, Elleray and Nordin 2 In cases with no lymph node involvement the 5 year survival is 80%, however, this falls to 50% with inguinal node involvement and 10–15% with iliac and pelvic node involvement. 3 Treatment modalities for cancer of the vulva are surgery, radiotherapy and chemotherapy, each having a significant role to play. Many patients diagnosed and treated for cancer of the vulva face major physical, social, sexual and psychological challengesReference Corney, Everett, Howells and Crowther 4 – Reference Warren, Melrose, Brooker and Burney 8 and studies show a need for more research into these issues.Reference Jefferies and Clifford 5 , Reference Likes, Stegbauer, Hathaway, Brown and Tillmanns 9 The rarity of cancer of the vulva means that many practitioners, including therapy radiographers, have little experience of meeting the psychosocial support needs of these patients. This fact along with the findings from a recent review highlighting a lack of published research into the topic,Reference Jefferies and Clifford 5 prompted a need to carry out a review of the psycho-social issues experienced by this patient group. This paper, by identifying recent studies and critically evaluating the available evidence, aims to present implications for practice suggesting ways to improve holistic person centred care for patients with cancer of the vulvar.
Method
A literature search was performed using Medline, Pubmed, CINAHL and PsycINFO. Keywords included vulva or vulval cancer, psychosocial, psychosexual impact and quality of life. Inclusion criteria consisted of publication between 1983–2017, written in the English language and available in full text. Types of studies included in the search were primary research studies, reviews and reports that included surgery for either cancer of the vulva or women diagnosed with precancerous condition vulval intraepithelial neoplasia (VIN), due to postoperative outcomes being similar.Reference Likes, Stegbauer, Hathaway, Brown and Tillmanns 9 Abstracts were reviewed for eligibility and articles were excluded if they focussed on cancers other than gynaecological and vulval cancers. A narrative approach to synthesis was used to integrate the evidence found and present findings as a narrative summary.Reference Dixon-Woods, Agarwal, Jones, Young and Sutton 10
Results
The search revealed numerous reports on the psychological and psychosocial problems faced by patients with gynaecological cancerReference Corney, Everett, Howells and Crowther 4 , Reference Tan, Waldman and Bostick 11 – Reference Aerts, Enzlin, Vergote, Verhaeghe, Poppe and Amant 13 together with articles on support or lack of support relating to this issue.Reference Sellick and Crooks 14 – Reference Bennion and Molassiotis 16 However, there was a paucity of literature specifically relating to cancer of the vulva as highlighted in previous reports.Reference Jefferies and Clifford 5 , Reference Janda, Obermair and Cella 17 – Reference Philp, Mellon, Barnett, D’Abrew and White 19 Cervical cancer was the most dominant diagnosis identified in the search with just 15 papers focusing specifically on cancer of the vulva. A summary of the key findings are presented below.
Psychological effects
Since cancer of the vulva is a rare condition, with limited research and a lack of studies into the impact of the disease, there is little known about the specific emotional, social and psychological impact for this patient group.Reference Jefferies and Clifford 5 , Reference Senn, Gafner and Happ 20 The psychological effects of being diagnosed with cancer can be felt long after the treatment has finished. 21 Surgery ranging from local excision to radical vulvectomy and removal of the clitoris can have a significant negative, psychological effect on these women.Reference Jefferies and Clifford 6 , Reference Senn, Gafner and Happ 20 , Reference Jefferies and Clifford 22 Symptoms may range from anxiety and sexual dysfunction to major depressive disorders. The onset of lower lymphoedema may also negatively affect body image and self-esteem with lymphoedema being a particularly distressing visible reminder of the disease.Reference De Hullu and Van der Zee 23 In addition, a report by Stellman et al.Reference Stellman, Goodman, Robinson, Dansak and Hilgers 24 states that four out of nine women studied were unable to name the area that was removed during surgery and suggests that this may have increased their feeling of embarrassment and isolation as they were unable to use the correct words to describe the area of their body affected by the diagnosis and treatment. The report recommends that patients undergoing vulvectomy should receive detailed information before and after surgery, and psychosexual counselling should be offered to both patient and her sexual partner on a long-term basis. Psychosocial distress in women diagnosed with cancer is outlined in a study by Warren et al.Reference Warren, Melrose, Brooker and Burney 8 Here researchers investigated the lived experience of patients in order to understand the underlying factors to psychosocial distress, using a qualitative research design to elicit and interpret women’s narratives of gynaecological cancer. The study revealed the role of social support, or its absence and recommends that interventions to address concerns may lead to improved quality of life. Poor perceived social support is also associated with increased distress for women with gynaecological cancer.Reference Hipkins, Whitworth, Tarrier and Jayson 25
Physical impact
The treatment modalities for cancer of the vulva although effective can impose severe disfigurement to the external genital area and this can have a significant impairment on patients’ quality of life.Reference Janda, Obermair and Cella 17 Other problems include bladder and bowel incontinence, vaginal dryness, stenosis, dyspareunia, lymphoedema, fatigue, all of which can have negative psychological reactions.Reference De Hullu and Van der Zee 23 – Reference Barlow, Hacker, Hussain and Parmenter 27 Janda et al.Reference Janda, Obermair and Cella 28 found that physical morbidity had a significant effect on daily life including on some women’s careers. The onset of lymphoedema is associated with groin node dissection and this can inhibit mobility and reduce physical strength.Reference Hadwin, Petts and Olaitan 29 Restrictions in activity resulting from surgery can also significantly contribute to a negative psychological impact.Reference Janda, Obermair and Cella 17 , Reference Hadwin, Petts and Olaitan 29
Sexuality and sexual functioning
The physical effects from the treatment of vulvar cancer can have a profound negative impact on sexual function, body image and sexuality. Surgery can be extensive and may require vulvectomy, resulting in damage to the vaginal introitus, and or removal of the clitoris.Reference Hadwin, Petts and Olaitan 29 A literature review by Aerts et al.Reference Aerts, Enzlin, Vergote, Verhaeghe, Poppe and Amant 13 found evidence that women who undergo surgical treatment for vulvar cancer or VIN are at high risk of sexual dysfunction, partner relationship problems and psychological issues. The authors concluded that due to a paucity of research regarding the impact of surgical treatment on quality of life and sexual function, there was a need for more methodologically sound research exploring these issues, together with studies into the functional relationship of both patient and their partners.
Discussion
The evidence identified above highlights the physical, sexual and psychological impact that the diagnosis and treatment of cancer of the vulva can have on patients. It also highlights, as with previous studies, the distinct lack of research into the psychosocial impact of this rare condition. A literature review carried out by Jefferies and Clifford,Reference Jefferies and Clifford 5 examined the psychological, physical and sexual consequences for women following a diagnosis and treatment for cancer of the vulva. The review gathered evidence between 1983 and 2007, and in total 14 studies reported in 15 papers. The study concluded a clear need for further research into issues for women facing treatment for carcinoma of the vulva. The authors also reported finding only one study involving British women.Reference Jefferies and Clifford 5 A similar review by Aerts et al.Reference Aerts, Enzlin, Vergote, Verhaeghe, Poppe and Amant 13 examined literature on the impact of surgical treatment for vulvar malignancy on sexual functioning, overall quality of life and partner relationships. They concluded that surgery had a negative impact on all the issues mentioned, although at the time of study there was only a small part of the study directed at the postoperative sexual wellbeing in vulvar cancer survivors and therefore authors acknowledged a more methodologically sound research was needed. Both these reviews had similar aims and both declared a paucity of literature, likely due to the rarity of the disease. Interestingly, a study by Green et al.Reference Green, Naumann, Elliot, Hall, Higgins and Grigsby 30 found that some women who were married or in a sexual relationship reported no significant difference in how they viewed the quality of the relationship after surgery, despite any decrease in or no sexual activity. This highlights the importance of looking at the broader view of sexuality for patients and their partners, rather than focussing on coital activity alone.Reference Sacerdoti, Lagana’ and Koopman 15 Jefferies and Clifford went on to publish a phenomenological study about the lived experience of 13 British women with cancer of the vulva and posed the question, ‘What must it be like to be diagnosed with and have surgery for a cancer of the vulva?’Reference Jefferies and Clifford 22 The study resulted in the women’s lived experience being described by its ‘invisibility’. Invisibility from lack of awareness, no one can see it, no one talks about it, no one has heard of it and invisibility, or lack of support. This is illustrated in Eve’s statement ‘I think if you had breast cancer or something like that, I think they (my friends) would be different because everybody knows about it, but with this sort of thing nobody knows about it’.Reference Jefferies and Clifford 22 , p. 387
This echoes women’s responses in semi-structured interviews carried out in a study by Warren et al.,Reference Warren, Melrose, Brooker and Burney 8 who went on to report the difficulties women had finding the appropriate services for their ‘forgotten cancer’, and how this left the women feeling excluded from a wider community of support, that they had the ‘wrong’ female cancer. Clarifying the meaning of the ‘wrong’ female cancer, these women were comparing the lack of information and support services available for cancer of the vulva to the amount of information that was available for breast cancer. Similar reports were found in a study by Senn et al.,Reference Senn, Gafner and Happ 20 where 20 Swiss and German patients were interviewed, regarding their experiences and social perceptions, in the first 6 months following surgical treatment for vulvar neoplasia. The main issues found were stigma with regards to the location of disease and a lack of information on what to expect and how to care for the vulva, leading to negative emotional responses, relationship problems and a cause of potentially unnecessary distress. The stigma associated with cancer of the vulva and the notions of women’s sexual propriety, affecting their willingness to talk about these issues and therefore increasing their sense of embarrassment and isolation, is also corroborated in studies by Wray et al.Reference Wray, Markovic and Manderson 18 and Jefferies and Clifford.Reference Jefferies and Clifford 6
Along a similar theme, an Australian study by Philip et al.Reference Philp, Mellon, Barnett, D’Abrew and White 19 aimed to explore and describe 12 women’s experience of diagnosis, treatment and recovery for vulvar cancer. They reported on participants of isolation, lack of information, lack of support and being unprepared psychologically for the physical changes in body image. These findings are also supported by the works of Senn et al., Jefferies and Clifford, and Warren et al.Reference Warren, Melrose, Brooker and Burney 8 , Reference Senn, Gafner and Happ 20 , Reference Jefferies and Clifford 22 Limitations due to the small sample size in the study by Philip et al. again reflects the rarity of the disease. Conversely, this low incidence of cancer of the vulva can also be a factor leading to delays in reporting and obtaining a diagnosis.Reference Mossman and Slevin 31 Studies show a significant negative, physical, psychological and sexual impact experienced by women diagnosed and treated with cancer of the vulva, a common theme being isolation, loneliness and stigmatisation due to lack of information not only for the patients but the carers too.Reference Warren, Melrose, Brooker and Burney 8 , Reference Philp, Mellon, Barnett, D’Abrew and White 19 , Reference Senn, Gafner and Happ 20 , Reference Jefferies and Clifford 22
Collaborative approach to management
Recommendations affirming the patients need for more detailed information before and after vulvectomy, for more support during and after treatment, and support in managing both the physical and psychosocial effects can be seen in studies as far back as Stellman.Reference Stellman, Goodman, Robinson, Dansak and Hilgers 24 Yet it is highlighted in studies as recent as 2017, more than three decades on, that patients continue to experience significant distress.Reference Philp, Mellon, Barnett, D’Abrew and White 19 Publications earlier than 2000 may not reflect current health care, however, they still hold relevance today. Recent studies consistent with the patients’ needs have explored women’s feelings and experiences,Reference Warren, Melrose, Brooker and Burney 8 , Reference Jefferies and Clifford 22 revealing that it is essential to listen to the voices of women with cancer of the vulva if we are to help with the specific needs of this particular group of women. It is well documented that the role of the Clinical Nurse Specialist (CNS) is crucial to the assessment of patients’ needs, offering support and care where appropriate.Reference Allen 32 , Reference Hood 33 Allen outlined the role of the CNS, in caring for the gynaecological patient, describing how they were best placed to help with the challenges and needs these patients face, to deliver appropriate information and aid with ‘quality of life issues’.Reference Allen 32 However, the studies outlined above suggest that there is still a lot more that can be done and there is a need for a more proactive multidisciplinary approach. Collaborating across professions is essential and there is a need for evaluation of current services exploring the potential for communication across teams involving other professionals in similar supportive roles, such as the specialised gynaecological treatment review radiographer (TRR). Given the growing interest in sentinel lymph node procedure and primary radiotherapy of the groins (as alternatives to inguinofemoral lymphadenectomy and chemoradiation, with or without surgery, as the first choice for patients with locally advanced squamous carcinoma of the vulva, only when primary surgery will necessitate performance of a stoma), it is important to consider the support needs for these patients.Reference De Hullu and Van der Zee 23 The TRR is uniquely placed in that they are available to provide effective face-to-face communication with the patient each day while attending for radiotherapy. This enables a helping relationship to be developed with the patient and provides an opportunity to identify concerns and to offer emotional support. 34 This impromptu intervention means that any treatment toxicities or concerns are dealt with in a timely manner, encouraging the patient to engage in caring for her genitalia, ensuring they are better informed and facilitating a prompt referral for psychological support where necessary. Providing reassurance and confidence for self-care is important and essential to patient recovery, along with ensuring any concerns are addressed in a sensitive manner. Studies show that patients feel they have little or no information on wound care once their treatment has finished and this can have a significant psychological impact.Reference Senn, Gafner and Happ 20 , Reference Allen 32 Supporting self management strategies and encouraging patients to play a role in ongoing self-management is an essential part of a team approach to facilitating supportive self-management.Reference Turton and Cooke 35 The specialised gynaecological TRR is highly experienced and able to facilitate patient assessment, particularly relating to the impact of radiotherapy treatment and its side effects, but is also able to identify any physical, psychological and sexual concerns. This allows for timely referral and communication directly with the wider multidisciplinary team (MDT) ensuring patient support needs are met.
Managing the psychological consequences
As previously discussed, psychological distress is common and vulvar cancer patients can find themselves facing difficult and distressing challenges at every stage of the patient pathway. 34 Studies have shown some important issues of concern, the most recent by Philp et al.Reference Philp, Mellon, Barnett, D’Abrew and White 19 highlighting the impact that isolation has on recovery and quality of life after treatment. The study reports the distress caused by the side effects from the treatment and the impact this had on relationships, body image and sexual health, and patients raised such concerns whether they were in a relationship or not. Women also felt that their follow-up was more medically directed and focussed on cancer recurrence; this highlights the importance of identifying the patient agenda and focussing on the physical, psychosocial, social, spiritual and financial information needs of patients with cancer of the vulvar. 36 Pitceathly et al. reported that in newly diagnosed cancer patients, those who were considered at high risk for psychological distress and were given a brief psychological intervention, were less likely to develop anxiety or depressive disorder, compared with those who received the usual care.Reference Pitceathly, Maguire, Fletcher, Parle, Tomenson and Creed 37 They went on to suggest that psychological care does not have to be provided by a psychologist or a psychiatrists for it to be effective and that it can be delivered by healthcare providers who have had the appropriate training. This is consistent with National Institute for Clinical Excellence (NICE) guidance which advocates a four-tier model of psychological support designed for all healthcare professionals directly responsible for the care of people with cancer. 34 A study by Powell et Al.Reference Powell, Kneier, Chen, Rubin, Kronewetter and Levine 38 showed evidence that a brief psychosocial intervention may be of benefit to gynaecological cancer survivors. However, in contrast to this, a review by Flynn et al.Reference Flynn, Kew and Kisely 39 found insufficient evidence to support or contest the use of any interventions for psychosexual dysfunction after gynaecological cancer, declaring all studies were of poor methodological quality and a need for more studies of high methodological quality was required. Recommendations were made for researchers to focus on interventions that can be provided by existing members of the MDT treating women with gynaecological cancers. This is in line with recommendations by the National Cancer Survivorship Initiative, 40 for assessment, planning and interventions aimed at support for patients living with and beyond cancer. Identifying the most appropriate time to address patients’ needs can be challenging for the healthcare professional and the need for an individual approach to assessment and intervention is required.Reference Hood 33 TRRs review patients weekly to monitor treatment toxicities and provide the appropriate information and support. It is reasonable to suggest here that the TRR, appropriately trained, would be well placed in identifying the optimal time and context for the discussion of any psychosocial issues. In line with the NICE guidelines, the TRR can ensure concerns are identified and that psychological assessment leads to appropriate intervention or referral as necessary. In this context, the role of advanced communication skills cannot be underestimated and good communication influences wellbeing, pain control and psychological health.Reference Dale, Petrova, Munday, Koistinen-Harris, Lall and Thomas 41 Face-to-face communication is highly valued between patients and their carers and there is a close relationship between good communication and providing emotional support. 34 As demonstrated in recent literature,Reference Philp, Mellon, Barnett, D’Abrew and White 19 , Reference Jefferies and Clifford 22 Warren et al. highlighted that women living with vulvar cancer have a sense of being alone, and emphasise the value of listening to women’s voices.Reference Warren, Melrose, Brooker and Burney 8 It is important to provide an opportunity for patients to understand the meaning of their lives when living with gynaecological cancer. These most recent studies exploring the existential wellbeing of patients living with vulvar cancer, underline the key issues these women are experiencing and underline how important it is to provide appropriate psychological support. Listening to the narratives of patients with cancer of the vulva is fundamental in order to understand how to care for them and to facilitate change.
Implications for clinical practice
In order to meet the psychological support needs for patients with cancer of the vulva and to enable participation in supportive self management, it is important that psychological assessment and intervention is timely and appropriate. Communication should be empathic and information needs assessed sensitively acknowledging the challenges faced by this patient group. The specialist gynaecological review radiographer is in a prime position to be able to assess patients’ information and support needs and ensure that appropriate information is provided. Such a role can provide face-to-face communication and information for patients, psychosocial support, clinical expertise, access to the wider MDT and research.
Therapy radiographers working with vulvar cancer patients need to provide a collaborative team approach, working alongside clinical psychologists and psychosexual counsellors to ensure psychosocial support needs are met. It is essential that good referral mechanisms are in place in line with the four-tier structure advocated by NICE. 34
As a majority of patients with vulvar cancer express feelings of isolation, and feel that they are the only one with this condition,Reference Jefferies and Clifford 6 , Reference Wray, Markovic and Manderson 18 , Reference Jefferies and Clifford 22 identifying mechanisms for peer support is an important area for development. Due to the rarity of cancer of the vulva, support groups may not be readily available, however, through the use of the Internet and multimedia, it is now possible to develop alternative methods of online support.
An important area for future consideration is patient education as there is a lack of information and awareness of the general public about female anatomy, the vulva and vulvar cancer. Providing information can help to raise awareness and encourage women to discuss their concerns. Information can be provided in a variety of settings including General Practitioner (GP) surgeries, well woman clinics and sexual health clinics. Posters and leaflets can improve awareness and ambiguity. Delivering information through sexual education in secondary schools, with more focus on the external genitalia, as well as the internal sexual organs may help improve the difficulties in discussing the female genitalia. Media advocacy and public narratives will raise the profile and diminish the stigma associated with vulvar cancer.
Finally given the limited amount of literature identified, it is essential that further research is carried out into the lived experiences of patients with cancer of the vulva, the psychological sequelae and the appropriateness of psychological interventions.
Conclusion
This paper has highlighted some important psychosocial issues faced by women diagnosed and living with cancer of the vulva, revealing many challenges experienced by this patient group. Challenges include social stigma surrounding the vulva and the diagnosis of vulvar cancer, feeling unprepared both physically and psychologically and a lack of information and support. It is fair to allude that these findings are attributed to limited research into the quality of life for these patients.Reference Janda, Obermair and Cella 17 , Reference Barlow, Hacker, Hussain and Parmenter 27 Consequently it is appropriate to suggest that further research, involving listening to women’s narratives, is essential if we are to provide appropriate support for this group of women. It is vital to gain an understanding of what is required to care for and educate them.
As the incidence for cancer of the vulva increases and especially in younger patients, this problem will be more frequently encountered and recent studies highlight the need to raise awareness among the general public and healthcare professions.
In the radiotherapy setting, specialist gynaecological radiographers are in a unique position to identify and manage concerns. Only by directly addressing these concerns can we provide better holistic patient centred care that supports the recovery of women who are diagnosed and treated with cancer of the vulva.
Acknowledgements
None.
Conflicts of Interest
None.
