Patient reported outcome measures among patients with vulvar cancer at various stages of treatment, recurrence, and survivorship.

OBJECTIVE: Our goal was to pragmatically describe patient reported outcomes (PROs) in a typical clinic population of vulvar cancer patients, as prior studies of vulvar cancer PROs have examined clinical trial participants. METHODS: A prospective PRO program was implemented in the Gynecologic Oncology clinic of a tertiary academic institution in January 2018. Vulvar cancer patients through September 2019 were administered the European Organization for the Research and Treatment of Cancer Quality of life Questionnaire, the Patient Reported Outcome Measurement Information System Instrumental and Emotional Support Scales, and the Functional Assessment of Cancer Therapy-Vulvar questionnaire. Binary logistic regressions were performed to determine adjusted odds ratios for adverse responses to individual questions by insurance, stage, age, time since diagnosis, recurrence, radiation, and surgical radicality. RESULTS: Seventy vulvar cancer patients responded to PROs (85.4% response rate). Seventy-one percent were > 1 year since diagnosis, 61.4% had stage I disease, and 28.6% recurred. Publicly insured women had less support and worse quality of life (QOL, aOR 4.15, 95% CI 1.00-17.32, p = 0.05). Women who recurred noted more interference with social activities (aOR 4.45, 95% CI 1.28-15.41, p = 0.019) and poorer QOL (aOR 5.22 95% CI 1.51-18.10, p = 0.009). There were no major differences by surgical radicality. Those >1 year since diagnosis experienced less worry (aOR 0.17, 95% CI 0.04-0.63, p = 0.008). CONCLUSIONS: Surgical radicality does not affect symptoms or QOL in vulvar cancer patients, whereas insurance, recurrence, and time since diagnosis do. This data can improve counseling and awareness of patient characteristics that would benefit from social services referral.

Quality of Life and Sexual Functioning After Vulvar Reconstruction With the Lotus Petal Flap.

OBJECTIVE: Resection of (pre) malignant lesions in the vulvoperineal area may result in large defects that cannot be closed primarily. The lotus petal flap technique is widely used for reconstruction. The aim of this study was to evaluate both quality of life (QoL) and sexual functioning of patients who underwent the lotus petal flap procedure, because no data are available on this topic. METHODS: A cross-sectional study was performed on all eligible patients (N = 38) who underwent the lotus petal flap procedure between 2005 and 2016. The European Organization for Research and Treatment of Cancer Quality of Life Questionnaire C30, Female Sexual Function Index, and Body Image Scale were used to evaluate QoL and sexual functioning. The European Organization for Research and Treatment of Cancer Quality of Life Questionnaire C30 and Female Sexual Function Index scores were compared with scores of age-matched healthy women. RESULTS: Twenty-six patients (68%) responded. The mean (SD) age was 65.5 (16.3) years, and the median follow-up time was 38.5 months (range 16-141 months). Quality of life scores were lower compared with healthy women in the domains physical, role, and social functioning. Sexual activity rates were comparable with healthy women; however, sexual functioning was worse. Although patients were satisfied about their sexual life, pain was reported. CONCLUSIONS: Patients who underwent vulvar reconstructive surgery with lotus petal flaps seem to have a lower QoL compared with healthy women. Patients report more pain during sexual activity but are satisfied about their sexual functioning. These results should be included in preoperative counseling and follow-up of future patients eligible for vulvar reconstruction with a lotus petal flap.

Review of the pathophysiology, diagnosis, and therapy of vulvar leiomyoma, a rare gynecological tumor.

The objective of this article is to discuss the pathophysiology, diagnosis, differential diagnosis, and therapy of vulvar leiomyoma. We performed a review of all English-language reports of vulvar leiomyoma published in PubMed from 1978 to 2015 using the following search terms: "vulval leiomyoma," "vulvar leiomyoma," "vulval smooth muscle tumor," and "external genitalia smooth muscle tumor." Vulvar leiomyomas, which are rare benign monoclonal tumors, most commonly occur in the fourth and fifth decades of life. The genetics of vulvar leiomyoma remain undefined. Three principal histological patterns have been identified: spindled, epithelioid, and myxoid. Imaging tests such as ultrasound, endoscopic ultrasound, and magnetic resonance imaging are used in diagnosis. Surgical excision is the only curative treatment for vulvar leiomyomas. Establishment of a full differential diagnosis list and correct final diagnosis before surgery are essential for optimal clinical management. Although recurrence of vulvar leiomyoma is extremely rare, long-term follow-up of all cases is advisable.

Clinicopathological features and differential diagnosis of aggressive angiomyxoma of the female pelvis: 5 case reports and literature review.

Aggressive angiomyxoma (AAM) is a rare mesenchymal tumor that usually occurs in the pelvis and perineum of young females. AAM can simulate Bartholin's gland cyst, abscess, lipoma, simple labial cyst, or other pelvic soft tissue tumors. Here we present five cases of AAM with mean age of 42. The patients mainly presented slow-growing mass in the abdomen and perineum (3 cases in the pelvis, 1 in the vulva, and 1 in the buttock). Color Doppler flow imaging revealed blood flow for the 3 pelvic lesions. Enhanced computed tomography and magnetic resonance imaging of the other 2 cases showed the typical "swirled" or "layered" structure characteristic. Through the pathological examination, its positivity to estrogen and progesterone receptors can justify enlargement and recurrence, confirming the tumor is AAM. All 5 patients underwent local tumor resection. Two patients recurred 8 and 15 months after surgery, respectively. The longest follow-up was 42 months. Although few cases are reported, early recognition demands high index of suspicion for both gynaecologists and pathologists. Wide surgical excision with tumor free margins is the basis of curative treatment. Adjuvant therapy may be necessary for residual or recurrent tumors. Long-term follow-up is recommended.

Carcinogenesis of Vulvar Lesions: Morphology and Immunohistochemistry Evaluation.

OBJECTIVES: The aim of the study was to assess the 2 pathways of vulvar carcinogenesis and correlate immunohistochemical expression of p53 with histopathological findings. MATERIALS AND METHODS: This cross-sectional study included 76 cases. Patients were classified according to the 2004 International Society for the Study of Vulvovaginal Disease Terminology, followed by a review of clinical records and immunohistochemical staining for p53. RESULTS: Fifteen cases were in the human papillomavirus (HPV)-associated pathway (12 cases of usual vulvar intraepithelial neoplasia [VIN] and 3 of warty squamous cell carcinoma [SCC]), and 13 cases were in the HPV-independent pathway (5 cases of differentiated VIN and 8 of keratinizing SCC). Significant differences in p53 expression were observed between the 2 pathways of carcinogenesis: in the lesions related to the HPV-independent pathway, the percentage of p53-positive cells was greater (>25%, p < .001), and the staining pattern was basal (extending into the middle layer) in differentiated VIN and diffuse or infiltrative in warty SCC (p < 0.001). In the lesions HPV-associated pathway, p53 staining was less extensive (≤10% of cells, p < 0.001) and followed basal pattern in usual VIN, whereas warty SCCs were negative for p53 (p < 0.001). CONCLUSIONS: Unique patterns of histological appearance and p53 expression can separate vulvar lesions into 2 distinct pathways of carcinogenesis. We propose that p53 immunohistochemistry may be performed simultaneously with histopathological examination in all cases of VIN and vulvar SCC, because it would aid in definition of the pathway of carcinogenesis and thus enable better clinical follow-up of patients with these conditions.

Sexual activity and function after surgical treatment in patients with (pre)invasive vulvar lesions.

PURPOSE: Sexual activity (SA) and sexual function (SF) are central outcome measures in women affected by preinvasive (vulvar intraepithelial neoplasia, VIN) and invasive (vulvar cancer, VC) vulvar lesions. Data on sexuality after treatment are scarce. METHODS: Validated questionnaires including the female sexual function index (FSFI-d) were provided to 166 women with a history of VIN and VC who attended the colposcopy units of the University Medical Center Hamburg-Eppendorf and Asklepios Medical Clinic Altona for follow-up between March 2011 and June 2012. Additional patients (n = 14) assessed the questionnaires online through the website of the German Vulvar Cancer Support Group (VulvaKarzinom SHG e.V.) during the same time period. RESULTS: Twenty-four patients with VIN and 34 with VC were evaluable. Median age was 51.5 years, with 34 (58.6%) of the patients being postmenopausal. Median time since completion of treatment was 17 months. All women had undergone vulvar surgery (laser/cold knife/combination). Overall, 14 (24.1%) women reported no SA during the last 4 weeks. SF was clearly impaired compared with previously described normal cohorts. SA and SF of active patients did not differ significantly between those with VIN and VC. Analyses contrasting surgical treatment methods yielded no significant associations; likewise, time since diagnosis did not affect SA and SF significantly. Increasing age was negatively associated with most dimensions of the FSFI-d [desire (p = 0.011), arousal (p = 0.004), lubrication (p = 0.003), orgasm (p = 0.013), satisfaction (p = 0.345), pain (p < 0.001)]. CONCLUSION: Women with VIN and VC after surgical treatment are at high risk to suffer from persistent sexual dysfunction especially at higher age.

Negative pressure wound treatment (NPWT) in vulva and groin wounds in gynaecologic oncology.

Secondary healing of complicated vulvar and groin wounds is a major challenge due to its moist condition and at risk of contamination by colonic flora. Vacuum assisted closure is the controlled application of sub-atmospheric pressure to the local wound environment using a sealed dressing connected to a vacuum pump. MATERIALS AND METHODS: The NPWT consists of an open-pore polyurethane ether foam sponge, an adhesive cover, fluid collection system, and suction pump that generates negative pressure. Direct application of sponge to blood vessels, bone, nerves or intact skin is avoided. The dressing and tubing are changed every 48-72 hours. RESULTS: Eight patients had NPWT following the vulva and/or groin surgery. Pain during removal of the sponge was the main adverse event requiring narcotic analgesia. All wounds healed completely. One patient is dead of disease progression. Others are alive without disease at four to 48 months. CONCLUSION: Wound breakdown in vulvar and groin surgery is an infrequent occurrence because of the rarity of full radical excision for vulva cancer and infralevator pelvic exenterative surgery. The present experience with NPWT was favourable.

Long-term sexual function in survivors of vulvar cancer: a cross-sectional study.

OBJECTIVES: To assess sexual function of vulvar cancer survivors who received extensive and less extensive treatment. To explore associations between sexual function and patient, disease, treatment, and psychological variables. METHODS: Sexual function (Female Sexual Function Index, FSFI), mental and physical well-being (SF36 Health Survey), body image (Body Image Scale), and optimism (Life Orientation Test) were assessed in vulvar cancer survivors treated in the period January 1997-January 2007. Demographic, disease and treatment characteristics were collected from medical files. Radical local excision with inguinal lymph node dissection and radical vulvectomy were considered extensive treatments; radical local excision, with or without sentinel node dissection, was considered less extensive treatment. Univariate and multivariate linear regression analyses were performed. RESULTS: Of 120 eligible patients, 76 (63%) responded. Eighteen women with a male partner (43%) reported having sexual intercourse. FSFI domain scores did not differ between extensively and less extensively treated women. Age was negatively associated with "Arousal" and "Desire", having a partner was positively associated with "Satisfaction", and optimism and physical well-being were positively associated with "Desire" and "Orgasm". Adjuvant inguinal radiotherapy was negatively associated with "Orgasm". One woman reported having better sexual function after than before treatment, 50% reported a similar sexual function, and 42% a worse sexual function. CONCLUSIONS: 43% of women who survived vulvar cancer and who had a male partner were sexually active. Treatment-related variables had a limited influence on long-term sexual function in these patients. Having a partner, good physical well-being, and being optimistic were positively associated with sexual function.

[Quality of life, social and psychological rehabilitation of surgical patients with vulvar cancer].

A questionnaire was used to evaluate quality of life of surgical patients with vulvar cancer (stages Ia-Iva). Overall and 5-year survival after combined and surgical treatment (combination of epidermato-fascial plasty unrestricted by closure of wound defects with adjacent tissues) was 86.05 +/- 3.2%, relapse-free--97.3% and 45.08 +/- 3.5% 57.1 +/- 4.1%, respectively, (p < or = 0.05) in group 1. Screening showed that surgical patients need more as far as quality of life and social rehabilitation are concerned.

Vulvar cancer as a target for molecular medicine.

Vulvar carcinoma is a rare female genital neoplasm. Although numerous molecular defects in vulvar carcinomas have been reported until now, no molecular markers that could be applied in daily clinical work have been identified so far. However, there is emerging evidence that specific mutations and gene expression patterns may be used as diagnostic tools in oncology. In this article we systematically review genetic alterations that may contribute to the development and progression of vulvar carcinoma. We conclude by suggesting molecular markers of potential clinical value in the diagnostics of this type of cancer.

A multidisciplinary approach to fertility-sparing therapy for a rare vulvar tumor.

OBJECTIVE: Case report of a young woman with a rare vulvar malignancy who received treatment with a personalized multidisciplinary approach to balance management of her malignancy without compromising survival with her desire for future pregnancy. DESIGN: Case report. SETTING: University hospital. PATIENT(S): A 33-year-old woman, gravida 2, para 1-0-1-1, who had a diagnosis of synovial cell sarcoma of the vulva and who desired future fertility. INTERVENTION(S): At multiple steps, treatment was planned to try to maximize fertility preservation without potentially affecting initial treatment, which included a radical hemivulvectomy with bilateral lymph node dissection, brachytherapy with interstitial needles (20 Gy), and external beam radiation therapy (50 Gy). MAIN OUTCOME MEASURE(S): Treatment and eradication of the malignancy and achievement of a successful subsequent pregnancy and live birth. RESULT(S): The patient had no evidence of disease for 2 years after treatment. During that time she received preconception counseling by both a perinatologist and a reproductive endocrinologist. She conceived with the use of ultrasound monitoring to time intercourse specifically with ovulation from the contralateral ovary and had an uncomplicated pregnancy with a term delivery. CONCLUSION(S): By using several disciplines and subspecialists, this patient received personalized treatment for a rare cancer, focused at curing her cancer and optimizing her future fertility.

Chlamydia trachomatis infection in women with lichen sclerosus vulvae and vulvar cancer.

OBJECTIVE: Chronic infections in the urogenital area often precede or coexist with vulvar cancer. A strong connection between some tumours and the-appearance of Chlamydia trachomatis infection has been observed, but there is little information concerning a connection of that infection with vulvar cancer and lichen sclerosus vulvae (LS). The aim of this study was the analysis of frequency of antigens appearance and antibodies of IgM and IgG Chlamydia trachomatis in patients with vulvar cancer and LS and we wanted to find the correlation between Chlamydia trachomatis infection and vulvar cancer and LS. METHODS: 80 women treated in the Clinic of Vulva Diseases at the Department and Clinical Ward of Gynaecology, Obstetrics and Oncological Gynaecology in Bytom, in the Silesian Medical University in Katowice were divided into two groups - 30 were treated for vulvar cancer and 50 were treated because of LS. We took bacterial smears vagina and cervical smears for presence of Chlamydia trachomatis antigens and peripheral blood to mark antibodies of IgM and IgG Chlamydia trachomastis. RESULTS: Chlamydia trachomatis antigen was found in 20% women with vulvar cancer and in 12% women with LS (p>0.05). In 13,3% cases with vulvar cancer we observed IgM Chlamydia trachomatis antibodies. In the group with LS IgM antibodies appeared in 16% women (p>0.05). In 50% patients with vulvar cancer in blood serum we observed IgG Chlamydia trachomatis antibodies, and in 16% women with LS (p<0.001). CONCLUSIONS: Previous Chlamydia trachomatis infection can lead to vulvar carcinogenesis.

Contribution of demographic, psychological and disease-related factors to quality of life in women with high-grade vulval intraepithelial neoplasia.

OBJECTIVES: To quantify the effect of demographic, psychological and disease-related factors on Quality of life (QoL) outcomes in women with high-grade vulval intraepithelial neoplasia (VIN2-3). To obtain qualitative data on the effect of disease and treatment in these women and their partners. To assess the participants' perception of their risk of developing of vulval cancer and its relation to QoL outcomes. METHODS: A questionnaire was constructed using existing instruments to measure the effect of demographic, psychological and disease-related factors on QoL outcomes. Free text space was provided for qualitative data. The questionnaire was mailed to women attending two specialist VIN clinics. RESULTS: One hundred and fifty women were invited for the study. Eighty-two responded (54.6%) of which forty-four (53.6%) were sexually active. Demographic factors (age and or living situation) had a significant effect on emotional health and body image. Psychological factors (anxiety and depression) had a significant effect on all aspects of QoL. Disease-related factors did not have a measurable effect on QoL outcomes, although the qualitative data revealed that various aspects of VIN had affected the lives of these women and their partners. There was a significant positive association between a perceived risk of developing vulval cancer with worsening general and emotional health. CONCLUSION: Psychological co-morbidity and various demographic factors should be considered while managing women with VIN. Accurate information regarding the development of vulval cancer should be given. The findings of this preliminary study will assist the construction of VIN-specific QoL instruments in the future.

Urologic function and urodynamic evaluation of urinary diversion (Rome pouch) over time in gynecologic cancers patients.

OBJECTIVE: To describe the urologic late complications and urodynamics outcome adopting teniamyotomies technique to create a low-pressure reservoir using the cecum, ascending colon and proximal part of the transverse colon without detubularization (Rome pouch). METHODS: Twenty-eight consecutive patients affected by gynecological cancer and submitted urinary diversion with "Rome pouch" technique were included. After 3 and 12 months from the surgical procedure patients were submitted to urodynamic evaluation of the neobladders. Excretory urography was performed in all patient. Abdominal X-ray, serum electrolytes, creatinine and cultures of the reservoir are obtained during every visit. Long-term urologic complications were recorded. Patient quality of life was assessed using a 10 cm grade visual analog scale (VAS). RESULTS: Urodynamics performed 12 months postoperatively showed that the mean maximum reservoir capacity was 439.9+/-58.9 cm H(2)O. The mean reservoir pressure at maximum capacity was 19.2+/-8.4 cm H(2)O (no contractive wave during the filling in any patient). The mean maximum closure pressure in the efferent tube, at maximum capacity, was 88.8+/-32.3 cm H(2)O. Continence was excellent for 26 (93%) and 23 (92%) patients at 3 and 12 months respectively. A total of 9 (32%) and 6 (24%) patients suffered late complications at 3 and 12 months follow-up respectively. However only one patient with pouch leakage underwent surgical pouch revision. CONCLUSION: Our experience demonstrated that Rome pouch creation with multiple teniamyotomies has good capacity with low internal pressure and good continence with a low rate of late urologic complications. Thus, comparing results to those of other continent pouch models, the Rome pouch technique represents a valid alternative.

Vulvar intraepithelial neoplasia: aspects of the natural history and outcome in 405 women.

OBJECTIVE: To determine the clinical characteristics, outcome following surgical treatment, and natural history of cases of untreated vulvar intraepithelial neoplasia (VIN). METHODS: Four hundred five cases of VIN 2-3 seen between 1962 and 2003 were reviewed. RESULTS: The mean age of women with VIN decreased from 50.0 years before 1980 to 39.0 in subsequent years. After treatment of VIN by excision and/or laser vaporization, half the women required at least one further treatment by the 14th year. It is estimated that 50% of women with positive surgical margins had at least one further treatment within 5 years, but only 15% of women with negative surgical margins required further treatment. Invasive vulvar, perianal, or urethral carcinoma occurred in 17 (3.8%) women (mean age 42 years) after treatment. Nine (2%) cases represented treatment failure, with a median treatment-to-invasion interval of 2.4 years. Eight (1.8%) cases represented new "field" carcinomas, with a median initial treatment-to-invasion interval of 13.5 years. Ten untreated cases, aged 32-76 years (mean age 55 years), progressed to invasion in 1.1-7.3 years (mean 3.9 years). Age, previous pelvic radiotherapy, unifocality, immunosuppression, and association with multicentric neoplasia were not risk factors for the development of invasive vulvar carcinoma in this study. Spontaneous regression of VIN occurred in 47 women (mean age 24.6 years), with a median interval to complete regression of 9.5 months. CONCLUSION: Surgically treated VIN has a high rate of recurrence. Untreated VIN in women over 30 years has an appreciable invasive potential.