The vulva is the area of the external sex organs of a woman. It is made up of two outer lips (the labia majora), which are covered in pubic hair and surround two inner lips (the labia minora). Between these lips are the entrances to the vagina and the the urethra (the short tube that passes urine from the bladder). Cancer of the vulvar (known as vulval or valvar cancer) occurs where the cells of the vulva become abnormal and grow in an uncontrolled way. There are a number of different types of cancer of the vulva. Most (about 90%) are squamous cell carcinoma which develop in the flat squamous skin cells. Less common cancers of the vulva include vulval melanoma, adenocarcinoma, and verrucous carcinoma. Paget’s disease of the vulva is a pre-cancerous condition where glandular cells spread outwards and across the vulval skin.
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MeSH term: Vulvar Neoplasms
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This list of publications is regularly updated (Source: PubMed).
Adenoid Cystic Carcinoma of Bartholin's Gland: Case Report.
Acta Med Iran. 2016; 54(12):820-822 [PubMed] Related Publications
Vaccines against human papillomavirus infections: protection against cancer, genital warts or both?
Clin Microbiol Infect. 2016; 22 Suppl 5:S125-S127 [PubMed] Related Publications
Immunological and Clinical Impact of Cancer Stem Cells in Vulvar Cancer: Role of CD133/CD24/ABCG2-Expressing Cells.
Anticancer Res. 2016; 36(10):5109-5116 [PubMed] Related Publications
MATERIALS AND METHODS: Paraffin-embedded tissue specimens derived from 43 patients with vulvar cancer were analyzed by immunohistochemistry for the expression of prominin-1 (CD133), CD24, ATP-binding cassette sub-family G member 2 (ABCG2) (CSC markers) and forkhead box protein P3 (FOXP3) (Treg marker).
RESULTS: CD133 expression correlated with younger age at diagnosis (p<0.01), lymph-node metastasis (p<0.05) and larger tumour diameter (p<0.05). CD133(+) tumours showed a high FOXP3(+) T-cell infiltration. Overall survival and progression-free survival were not influenced by the expression of the analyzed biomarkers.
CONCLUSION: In vulvar cancer, CSCs were more frequently expressed in younger aged patients and those with aggressive disease. Their presence was also associated with high Treg infiltration, which contributes to the generation of an immunosuppressive milieu.
Clitoral Metastasis From Advanced Cervical Carcinoma on 18F-FDG-PET/CT.
Clin Nucl Med. 2017; 42(1):54-55 [PubMed] Related Publications
Depth and Patterns of Adnexal Involvement in Primary Extramammary (Anogenital) Paget Disease: A Study of 178 Lesions From 146 Patients.
Am J Dermatopathol. 2016; 38(11):802-808 [PubMed] Related Publications
Surgical management for squamous cell carcinoma of vulva.
Pan Afr Med J. 2016; 24:145 [PubMed] Free Access to Full Article Related Publications
Risk factors and treatment for recurrent vulvar squamous cell carcinoma.
Crit Rev Oncol Hematol. 2016; 106:1-13 [PubMed] Related Publications
Medical and Surgical Treatments for Usual-Type Vulvar Intraepithelial Neoplasia.
JAMA Oncol. 2016; 2(12):1647-1648 [PubMed] Related Publications
Bottom Line: Provided cancer is not suspected, usual-type vulvar intraepithelial neoplasia treatment, including medical and surgical options, can be individualized to take into account the site, extent of disease, and a woman's preferences, with a commitment to long-term follow-up.
Vulvar and Vaginal Cancer, Vulvar Intraepithelial Neoplasia 3 and Vaginal Intraepithelial Neoplasia 3: Experience of a Referral Institute.
Isr Med Assoc J. 2016; 18(5):286-9 [PubMed] Related Publications
OBJECTIVES: To evaluate the demographic and clinical characteristics associated with vulvar or vaginal cancer and vulvar and vaginal intraepithelial neoplasia 3 (VIN3, VAIN3).
METHODS: We conducted a retrospective chart review of 148 women with vulvar and vaginal malignancy and pre-malignancy for the period October 2004 to October 2012, and identified 59 and 19 patients with vulvar and vaginal cancer respectively, and 57 and 13 patients with VIN3 and VAIN3 respectively
RESULTS: The median age of vulvar cancer patients was 30 years older than that of VIN3 patients. HPV was found in 60% and 66.6% of vulvar and vaginal cancer patients respectively, and in 82.3% and 84.6% of patients with VIN3 and VAIN3 respectively. A history of cervical intraepithelial neoplasia (CIN) or warts was observed in 10% and 10.5% of vulvar and vaginal cancer patients respectively, and in 57.9% and 46% of patients with VIN3 and VAIN3 respectively. In 52.6% of patients the vaginal cancer was metastases from other organs.
CONCLUSIONS: Most women with vulvar carcinoma are older than 70 years. VIN3 and VAIN3 are associated with HPV infection and the most prevalent type is HPV16. Almost half the vaginal cancers are associated with metastases from other organs and almost half of VAIN3 is associated with past cervical dysplasia or carcinoma.
The versatility of profunda femoral artery perforator flap for oncological reconstruction after cancer resection-Clinical cases and review of literature.
J Surg Oncol. 2016; 114(2):193-201 [PubMed] Related Publications
METHODS: A total of 55 free PAP flaps and 16 pedicle PAP flaps were transferred in 63 patients. Each case was reviewed to verify a PAP flap was performed identifying defect location, flap size, flap design, and postoperative complications.
RESULTS: Seven flaps in five patients underwent breast reconstructions, 48 patients underwent head and neck reconstructions using free PAP flaps. The mean perforator number was 1.9, and the average pedicles length was 9.7 cm. The majority of perforators were musculocutaneous, and the others were septocutaneous. The mean ischemia time was 121.4 min. Minor complications included wound poor healing, flap partial necrosis, and pedicle vessels problems. Sixteen pedicle PAP flaps were transferred in 10 patients for vulvar reconstruction. Minor complications included urinary tract infection, poor wound healing, wound infection, hematoma.
CONCLUSIONS: The anatomy and number of perforators of PAP flap are reliable with adequate pedicle length. This flap can be an excellent option for reconstruction of most soft tissue defects. J. Surg. Oncol. 2016;114:193-201. © 2016 Wiley Periodicals, Inc.
Stathmin is a highly sensitive and specific biomarker for vulvar high-grade squamous intraepithelial lesions.
J Clin Pathol. 2016; 69(12):1070-1075 [PubMed] Related Publications
METHODS: Immunohistochemical analysis was used to evaluate stathmin, P16 and Ki67 expression in 91 samples, including LSILs (n=16), HSILs (n=50), differentiated VIN (dVIN; n=10), lichen sclerosis (LS; n=10) and normal vulvar tissue (n=5).
RESULTS: Stathmin was expressed in more than one-third of the epithelium in all HSILs and in 20% of LSILs. P16 and Ki67 were expressed in more than one-third of the epithelium in 94% of HSILs and in 13% and 40% of LSILs, respectively. Stathmin was expressed in more than one-third of the epithelium in 10% of the dVIN and in none of the LS or normal lesions. P16 and Ki67 expression was not present in more than one-third of the epithelium in any of these lesions. The sensitivity of stathmin for differentiating between LSILs and HSILs was 100% compared with a sensitivity of 94% for both p16 and Ki67. The specificity of stathmin, p16 and Ki67 was 80%, 87% and 60%, respectively.
CONCLUSIONS: Stathmin is a highly sensitive and specific biomarker for the diagnosis of vulvar HSIL. In addition to the more commonly used immunohistochemical markers p16 and Ki67, stathmin can be a useful diagnostic tool for identifying HSILs, especially in cases in which differentiating between LSIL and HSIL is difficult.
FDG-PET/CT of Vulvar Adenocarcinoma With Diffuse Metastases.
Clin Nucl Med. 2016; 41(9):710-1 [PubMed] Related Publications
Radiotherapy for gynecologic cancer in nonagenarian patients: a framework for new paradigms.
Chin J Cancer. 2016; 35:43 [PubMed] Free Access to Full Article Related Publications
Determination of Oncogenic Human Papillomavirus (HPV) Genotypes in Anogenital Cancers in Myanmar.
Acta Med Okayama. 2016; 70(2):103-10 [PubMed] Related Publications
Treatment results and prognostic factors of patients with vulvar cancer treated with postoperative or definitive radiotherapy.
Tumori. 2016; 2016(3):311-5 [PubMed] Related Publications
METHODS: Forty-four vulvar cancer patients treated between 2000 and 2011 at the Department of Radiation Oncology, Ege University Faculty of Medicine, were retrospectively reviewed. External radiotherapy (RT) was applied with 6-18 MV linear accelerators with 1.8 Gy daily fractions with a median total dose of 50.4 Gy (45-59.4 Gy) for postoperative cases and 64.8 Gy (range 54-66 Gy) for definitive cases. Statistical analyses were performed with SPSS 13.0.
RESULTS: Among 44 patients with a median age of 68 years (range 28-86), 14 (31.8%) were treated with curative and 30 (68.2%) were treated with postoperative RT or radiochemotherapy (RCT). According to International Federation of Gynecology and Obstetrics staging, 11 (25%) had stage IB, 10 (22.7%) had stage II, 6 (13.6%) had stage IIIA, 5 (11.4%) had stage IIIB, and 12 (27.3%) had stage IVA disease. Within a median of 24 months (range 6-135) of follow-up, 11 (27.3%) patients had local recurrence, 8 had regional recurrence, 2 had both local and regional recurrence, and 6 had distant metastases. Five-year locoregional, disease-free, and overall survival rates were 45%, 40%, and 54%, respectively. Older age, poor tumor differentiation, positive surgical margin, and lymphovascular space invasion were found to be important prognostic factors for disease-related outcomes.
CONCLUSIONS: Prognosis of vulvar cancer remains poor even with a multidisciplinary approach. Molecular prognostic factors need to be defined for individualized treatment options to achieve better treatment results.
Profunda artery perforator flap for isolated vulvar defect reconstruction after oncological resection.
J Surg Oncol. 2016; 113(7):828-34 [PubMed] Related Publications
BACKGROUND: Vulvar reconstruction remains a great challenge to reconstructive surgeons. A local fasciocutaneous flap from the medial thigh is a good option with multiple choices of the donor arteries. Here, we extended the clinical application of a profunda perforator artery (PAP) flap with the design of an island pedicle flap.
METHODS: From 2012 to 2015, 12 female patients with vulvar cancer received tumor ablation and immediate reconstruction using a PAP flap. The flaps (n = 19) were divided into V-Y advancement perforator flap (group I, n = 4) and island pedicle perforator flap (group II, n = 15). All of the demographic data were collected and analyzed.
RESULTS: All of the flaps were transferred successfully, and all of the donor sites were closed without morbidities. Group II was superior to group I because of the smaller required flap size (P = 0.004), the smaller defect size/flap size ratio (P = 0.001), and a lower rate of post-op debridement (P = 0.037). The other parameters were not statistically significant.
CONCLUSIONS: PAP flap is a good choice for vulvar reconstruction. We preferred an island pedicle setting for its thin and pliable fasciocutaneous component and robust flap circulation. The favorable functional and aesthetic results can be achieved with limited donor site morbidities. J. Surg. Oncol. 2016;113:828-834. © 2016 Wiley Periodicals, Inc.
Aggressive Angiomyxoma of the Vulva with No Recurrence on a 5-year Follow up: A Case Report.
Tokai J Exp Clin Med. 2016; 41(1):42-5 [PubMed] Related Publications
Skin metastases of vulvar squamous cell carcinoma. Presentation of a rare case.
Eur J Gynaecol Oncol. 2016; 37(1):126-8 [PubMed] Related Publications
Neoadjuvant chemoradiotherapy followed by radical vulvectomy for adenoid cystic carcinoma of Bartholin's gland: a case report and review of the literature.
Eur J Gynaecol Oncol. 2016; 37(1):113-6 [PubMed] Related Publications
The mandatory role of groin lymphadenectomy in clinical Stages IB and II vulvar cancer.
Eur J Gynaecol Oncol. 2016; 37(1):86-8 [PubMed] Related Publications
MATERIALS AND METHODS: Twenty-two patients with FIGO Stages IB-II FIGO vulva cancer with no clinically and imagistic evidence of nodes metastases were treated in the present clinic. The surgical procedures consisted in radical vulvectomy plus inguinofemoral lym- phadenectomy.
RESULTS: The final pathological result was squamous carcinoma in 20 patients, vulva melanoma in one, and carcinosar- coma in one. The prevalence of positive lymph nodes was 45.4%. The median number of harvested lymph nodes was 14.0 per groin (between four and 27). Twelve patients (54.5%) developed some wound complications, but all were resolved. At the present time, 20 patients are alive, but the follow-up period is short for many of them; two patients died of disease.
CONCLUSION: The prevalence of groin metastases in Stages IB-II vulvar cancer is high. A thorough inguino-femoral dissection seems necessary, despite the high incidence of wound complications.
Genital invasion or perigenital spread may pose a risk of marginal misses for Intensity Modulated Radiotherapy (IMRT) in anal cancer.
Radiat Oncol. 2016; 11:53 [PubMed] Free Access to Full Article Related Publications
METHODS: We present and discuss four patients with perigenital spread in anal cancer in both early and advanced stages (three at time of first diagnosis and one in form of relapse). Genital/perigenital spread was observed once as direct genital infiltration and thrice in form of perigenital lymphatic spread.
RESULTS: We review the available data regarding the potential consequences of genital sparing in anal cancer. Pattern-of-relapse studies in anal cancer after conventional radiotherapy and the current use of IMRT in anal cancer are equivocal but suggest that genital sparing may occasionally result in marginal miss. An obvious hypothesis suggested by our report is that perigenital lymphovascular invasion might be associated with manifest inguinal N+ disease.
CONCLUSIONS: Local failure has low salvage rates in recent anal cancer treatment series. Perigenital spread may pose a risk of marginal misses in IMRT in anal cancer. To prevent marginal misses, meticulous pattern-of-relapse analyses of controlled IMRT-series are warranted. Until their publication, genital sparing should be applied with caution, PET/CT should be used when possible and meeting genital dose constraints should not be prioritized over CTV coverage, especially (but not only) in stage T3/4 and N+ disease.
Detection and Type-Distribution of Human Papillomavirus in Vulva and Vaginal Abnormal Cytology Lesions and Cancer Tissues from Thai Women.
Asian Pac J Cancer Prev. 2016; 17(3):1129-34 [PubMed] Related Publications
Prognostic Value of Overexpressed p16INK4a in Vulvar Cancer: A Meta-Analysis.
PLoS One. 2016; 11(3):e0152459 [PubMed] Free Access to Full Article Related Publications
METHODS: All publications in English language on the association between p16INK4a and clinicopathological features of vulvar cancer were searched from Pubmed, Embase, and Web of Science, and those in Chinese language were identified manually and online from the China National Knowledge Infrastructure. Strict inclusion and exclusion criteria were followed. Odds ratios (ORs) or risk ratios (RRs) with 95% confidence intervals (CIs) were pooled to assess the strength of association. Publication bias was estimated using funnel plots and the Egger's regression test.
RESULTS: A total of 17 studies with 2309 patients were included. The p16INK4a overexpression was found to correlate significantly with the lower International Federation of Gynecology and Obstetrics stage (I+II vs III+IV; OR = 0.60, 95%CI: 0.41-0.86, P = 0.006), negative lymph node metastasis(negative vs positive; OR = 0.61, 95%CI: 0.39-0.95, P = 0.029), patient's age <55 (OR = 0.54, 95%CI: 0.31-0.96, P = 0.034), human papillomavirus-positive status (OR = 0.01, 95%CI: 0.00-0.11, P<0.001), and higher overall survival (RR = 0.53, 95%CI = 0.35-0.80, P = 0.003).
CONCLUSION: The p16INK4a might be associated with a higher survival and indicates better prognosis of vulvar cancer.
Vulvar intraepithelial neoplasia grade 3 associated with Behçet's disease.
BMC Res Notes. 2016; 9:172 [PubMed] Free Access to Full Article Related Publications
CASE: A 44-year-old Caucasian woman with a history of BD, which had been evolving for 6 years, presented with ulcerated and papillomatous lesions on the vulva. Biopsies revealed a multifocal VIN3 positive for high-risk human papillomavirus (HPV) 33. Multiple biopsies were performed to exclude invasive cancer and VIN3 was treated with laser vaporization.
CONCLUSION: We report clinical and anatomopathological features of a rare case of multifocal, high-risk, HPV-related VIN3. We also discuss the possible pathogenesis in the context of BD, featuring chronic ulceration and intrinsic or treatment-induced immunosuppression.
Trends in surgery and outcomes of squamous cell vulvar cancer patients over a 16-year period (1998-2013): a population-based analysis.
J Cancer Res Clin Oncol. 2016; 142(6):1331-41 [PubMed] Related Publications
METHODS: A total of 1113 patients with squamous cell vulvar cancer diagnosed between 1998 and 2013 in the catchment area of the Munich Cancer Registry (population approximately 4.6 million) were analysed. Trends in prognostic factors and treatment were examined by comparing patients diagnosed between 1998 and 2008 with those diagnosed between 2009 and 2013. Cumulative incidence was used to calculate time to local (LR) and lymph node recurrence (LNR). Survival was analysed by the Kaplan-Meier method, calculation of relative survival (RS), and a Cox model.
RESULTS: The high median age at diagnosis of 75 years did not change significantly over time. In addition, no changes in the subsite of tumour or grading were noted. A decrease in patients undergoing complete vulvectomy from 27.7 to 17.8 % (p < 0.001) as well as an increase in the use of sentinel lymph node biopsy from 11.4 to 39.1 % (p < 0.001) was observed. However, time to LR (from 19 to 19 %) and time to LNR (from 9 to 9 %) as well as 5-year overall survival (from 55 to 55 %) and RS (from 66 to 63 %) were not significantly altered. After adjustment for prognostic factors, less radical locoregional surgery had no influence on survival.
CONCLUSION: Less radical locoregional surgery in vulvar cancer is increasingly implemented. Locoregional recurrence and survival have not been affected by these changes and are likely accompanied by an improvement in quality of life.
Paget disease of the vulva.
Crit Rev Oncol Hematol. 2016; 101:60-74 [PubMed] Related Publications
Primary Non-Hodgkin's Lymphoma of the Vulva: A Case Report and Literature Review.
Medicine (Baltimore). 2016; 95(10):e3041 [PubMed] Free Access to Full Article Related Publications
Dosimetric evaluation and clinical outcome in post-operative patients of carcinoma vulva treated with intensity-modulated radiotherapy.
Indian J Cancer. 2015 Oct-Dec; 52(4):670-5 [PubMed] Related Publications
MATERIALS AND METHODS: A total of 8 post-operative patients of vulvar cancer were treated with IMRT. All patients were also planned by 3DCRT for comparison with IMRT. The two plans were compared in terms of conformity index, homogeneity index, tumor control probability (TCP) and normal tissue complication probability (NTCP) for the planning target volume and organs at risk (OAR).
RESULTS: IMRT resulted in significantly lesser doses to rectum, bladder, bowel and femoral head as compared with 3DCRT plans. Mean conformity and homogeneity indices were better and within range with IMRT. The TCP was comparable between the two treatment plans and NTCP for rectum, bladder, bowel and femoral head was significantly less with IMRT as compared with 3DCRT. Treatment was well-tolerated and none of the patients developed Grade 3 or higher toxicity.
CONCLUSION: IMRT yielded superior plans with respect to target coverage, homogeneity and conformality while lowering dose to adjacent OAR as compared with 3DCRT. Thus, IMRT offers a reduction in NTCP while maintaining TCP.
Myoepithelioma-like Tumor of the Vulvar Region Presenting as a Nonmyxoid Spindle-Cell Neoplasm: A Potential Histologic Mimicker of Solitary Fibrous Tumor.
Am J Dermatopathol. 2016; 38(7):e87-9 [PubMed] Related Publications
Vulvar intraepithelial neoplasia: clinical presentation, management and outcomes in women infected with HIV.
AIDS. 2016; 30(6):859-68 [PubMed] Related Publications
DESIGN: Observational cohort study.
METHODS: Data was collected from women diagnosed with VIN at the Hospital Vall d'Hebron between September 1994 and October 2011. The main outcome measures were recurrence-free survival (RFS) and progression-free survival (PFS). Risk factors for recurrence and progression were assessed using univariate and multivariate analyses.
RESULTS: Thirty-seven out of 107 women were HIV positive (34.6%). The median follow-up time was 32 (range 12-179) months. Compared with the HIV-negative group, HIV-positive women were younger (median age 37 vs. 44 years, P = 0.003) and presented with multifocal and multicentric disease more frequently (63.6 vs. 22.2% and 84.8 vs. 43.3%, respectively, P < 0.0001). RFS and PFS were lower in the HIV-positive group (42.4 vs. 71.4% P = 0.043 and 69.7 vs. 95.2% P = 0.006, respectively). RFS was significantly associated to multicentric and multifocal disease on multivariate analysis. PFS was associated to HIV infection on univariate analysis.
CONCLUSION: HIV-positive women are at increased risk of developing VIN and frequently present at a younger age with multifocal and multicentric disease. They have shorter RFS and PFS compared with HIV-negative women. Close surveillance of the lower genital tract is mandatory to enable early recognition and treatment of any suspicious lesions. Close follow-up after treatment of VIN is essential to exclude early recurrence or progression.