Vulva Cancer
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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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Information for Patients and the Public
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Latest Research Publications

Information Patients and the Public (14 links)


Information for Health Professionals / Researchers (7 links)

Latest Research Publications

This list of publications is regularly updated (Source: PubMed).

Baiocchi G, Cestari FM, Rocha RM, et al.
Does the count after inguinofemoral lymphadenectomy in vulvar cancer correlate with outcome?
Eur J Surg Oncol. 2013; 39(4):339-43 [PubMed]
BACKGROUND: Inguinal lymph node (LN) metastasis is an important prognostic factor in vulvar cancer. Our aim was to determine the prognostic value of the number of resected LNs in inguinofemoral lymphadenectomy.
METHODS: A retrospective analysis was performed in a series of 158 individuals who underwent bilateral inguinofemoral lymphadenectomy for vulvar squamous cell carcinoma from January 1980 to February 2010.
RESULTS: The mean age was 67 years (range: 15-90). Median tumor size was 5 cm (range: 1-18). A median of 22.5 inguinal LNs (range: 2-57) was resected. Thirteen (8.2%) patients had <12 LNs resected, and 145 (91.8%) had ≥ 12 LNs resected. Eighty (50.6%) patients had LN metastasis, with a median of 2 positive LNs (range: 1-16). Of those with positive LNs, 19 (23.8%), 23 (28.8%), and 38 (47.5%) patients had 1, 2, and 3 or more positive LNs, respectively. Thirty-three (41.2%) patients had bilateral LN metastasis. For patients without LN involvement, we failed to observe any significant difference between patients with <12 LNs and ≥ 12 LNs that were resected with regard to risk of recurrence (p=0.97) and death from cancer (p=0.43) in 5 years. However, resection of <12 LNs in patients with positive LNs negatively impacted the risk of recurrence (p=0.003) and death from cancer (p=0.043).
CONCLUSIONS: Resection of fewer than 12 LNs in vulvar cancer has a negative impact on outcome for patients with positive inguinal LNs.


Gadducci A, Ferrero A, Tana R, et al.
Prognostic value of lymph node status and number of removed nodes in patients with squamous cell carcinoma of the vulva treated with modified radical vulvectomy and inguinal-femoral lymphadenectomy.
Eur J Gynaecol Oncol. 2012; 33(6):640-3 [PubMed]
PURPOSE OF INVESTIGATION: To assess the outcome of patients with squamous cell vulvar carcinoma treated with deep partial or total vulvectomy and inguinal-femoral lymphadenectomy.
MATERIALS AND METHODS: The authors assessed 87 patients who underwent primary surgery.
RESULTS: Tumor recurred in 34 patients, and the first relapse was local in 19, inguinal in ten, and distant in five. Five-year disease-free survival was 56.7% and was related to Stage (p < 0.0001), grade (p = 0.023), and node status (p < 0.0001). Groin failure occurred in 4.9% of node-negative patients compared with 29.6% of node-positive patients (p = 0.0096). Distant recurrences only developed in women with positive nodes. Among the 47 patients who underwent bilateral lymphadenectomy and who had negative nodes, groin recurrence occurred in 12% of those who had < or = 15 nodes removed and 0% of those who had > 15 nodes removed.
CONCLUSIONS: Stage and node status were the most important prognostic variables. There was a trend favoring a better groin control in patients with node-negative disease who underwent extensive lymphadenectomy.


Agu PU, Okeke TC, Ezugwu EC, Obi SN
Large vulvar lipoma following episiotomy: a case report.
Niger J Med. 2012 Jul-Sep; 21(3):357-8 [PubMed]
Vulvar lipomas are rare few cases have been reported in the world literature. We document a case of large soft vulvar mass following episiotomy in a 23-year-old primipara. The mass was excised and histologic examination confirmed lipoma.


Senn B, Eicher M, Mueller MD, et al.
A patient-reported outcome measure to identify occurrence and distress of post-surgery symptoms of WOMen with vulvAr Neoplasia (WOMAN-PRO) - a cross sectional study.
Gynecol Oncol. 2013; 129(1):234-40 [PubMed]
OBJECTIVE: The aim of the study was to describe the (a) symptom experience of women with vulvar intraepithelial neoplasia and vulvar cancer (vulvar neoplasia) during the first week after hospital discharge, and (b) associations between age, type of disease, stage of disease, the extent of surgical treatment and symptom experience.
METHODS: This cross-sectional study was conducted in eight hospitals in Germany and Switzerland (Clinical Trial ID: NCT01300663). Symptom experience after surgical treatment in women with vulvar neoplasia was measured with our newly developed WOMAN-PRO instrument. Outpatients (n=65) rated 31 items. We used descriptive statistics and regression analysis.
RESULTS: The average number of symptoms reported per patient was 20.2 (SD 5.77) with a range of 5 to 31 symptoms. The three most prevalent wound-related symptoms were 'swelling' (n=56), 'drainage' (n=54) and 'pain' (n=52). The three most prevalent difficulties in daily life were 'sitting' (n=63), 'wearing clothes' (n=56) and 'carrying out my daily activities' (n=51). 'Tiredness' (n=62), 'insecurity' (n=54) and 'feeling that my body has changed' (n=50) were the three most prevalent psychosocial symptoms/issues. The most distressing symptoms were 'sitting' (Mean 2.03, SD 0.88), 'open spot (e.g. opening of skin or suture)' (Mean 1.91, SD 0.93), and 'carrying out my daily activities' (Mean 1.86, SD 0.87), which were on average reported as 'quite a bit' distressing. Negative associations were found between psychosocial symptom experience and age.
CONCLUSIONS: WOMAN-PRO data showed a high symptom prevalence and distress, call for a comprehensive symptom assessment, and may allow identification of relevant areas in symptom management.


Tergas AI, Tseng JH, Bristow RE
Impact of race and ethnicity on treatment and survival of women with vulvar cancer in the United States.
Gynecol Oncol. 2013; 129(1):154-8 [PubMed]
OBJECTIVE: To examine racial/ethnic differences in treatment and survival in women diagnosed with invasive vulvar cancer in the United States.
METHODS: Women with invasive vulvar cancer were identified from the Surveillance, Epidemiology, and End Results database from 1/1/92 to 12/31/02. Statistical analysis using Chi-square, Fisher's Exact Test, Kaplan-Meier survival methods, and Cox regression proportional hazards models was performed.
RESULTS: Of the 2357 cases of invasive vulvar cancer included in this study, 1974 (83.8%) were non-Hispanic white, 209 (8.9%) were non-Hispanic black, 119 (5.0%) were Hispanic, and 55 (2.3%) women were of another race/ethnicity. After adjustment for stage, black women were half as likely (OR=0.48, 95% CI 0.31-0. 74) to undergo surgery and 1.7 times more likely (OR=1.67, 95% CI 1.18-2.36) to receive radiation than white women. In multivariable analysis, surgical treatment reduced the risk of death from vulvar cancer by 46% (HR 0.54, 95% CI 0.43-0.67), whereas radiation was not shown to impact the risk of death (HR 0.99, 95% CI 0.84-1.19), after adjusting for age, race, stage, and grade. There was no significant difference in risk of death by race/ethnicity group after adjusting for the previously described variables.
CONCLUSIONS: Based on this study, race/ethnicity is not an independent risk factor for poor prognosis in women diagnosed with invasive vulvar cancer, despite differences in treatment modality by race/ethnicity. Further research to define the factors contributing to differences in treatment selection according to race/ethnicity and the resulting impact on quality of life is warranted.


Beriwal S, Shukla G, Shinde A, et al.
Preoperative intensity modulated radiation therapy and chemotherapy for locally advanced vulvar carcinoma: analysis of pattern of relapse.
Int J Radiat Oncol Biol Phys. 2013; 85(5):1269-74 [PubMed]
PURPOSE: To examine clinical outcomes and relapse patterns in locally advanced vulvar carcinoma treated using preoperative chemotherapy and intensity modulated radiation therapy (IMRT).
METHODS AND MATERIALS: Forty-two patients with stage I-IVA (stage I, n=3; stage II, n=13; stage III, n=23; stage IVA, n=3) vulvar cancer were treated with chemotherapy and IMRT via a modified Gynecological Oncology Group schema using 5-fluorouracil and cisplatin with twice-daily IMRT during the first and last weeks of treatment or weekly cisplatin with daily radiation therapy. Median dose of radiation was 46.4 Gy.
RESULTS: Thirty-three patients (78.6%) had surgery for resection of vulva; 13 of these patients also had inguinal lymph node dissection. Complete pathologic response was seen in 48.5% (n=16) of these patients. Of these, 15 had no recurrence at a median time of 26.5 months. Of the 17 patients with partial pathological response, 8 (47.1%) developed recurrence in the vulvar surgical site within a median of 8 (range, 5-34) months. No patient had grade ≥3 chronic gastrointestinal/genitourinary toxicity. Of those having surgery, 8 (24.2%) developed wound infections requiring debridement.
CONCLUSIONS: Preoperative chemotherapy/IMRT was well tolerated, with good pathologic response and clinical outcome. The most common pattern of recurrence was local in patients with partial response, and strategies to increase pathologic response rate with increasing dose or adding different chemotherapy need to be explored to help further improve outcomes.


Olsen J, Jørgensen TR, Kofoed K, Larsen HK
Incidence and cost of anal, penile, vaginal and vulvar cancer in Denmark.
BMC Public Health. 2012; 12:1082 [PubMed] Free Access to Full Article
BACKGROUND: Besides being a causative agent for genital warts and cervical cancer, human papillomavirus (HPV) contributes to 40-85% of cases of anal, penile, vaginal and vulvar cancer and precancerous lesions. HPV types 16 & 18 in particular contribute to 74-93% of these cases. Overall the number of new cases of these four cancers may be relatively high implying notable health care cost to society. The aim of this study was to estimate the incidence and the health care sector costs of anal, penile, vaginal and vulvar cancer.
METHODS: New anogenital cancer patients were identified from the Danish National Cancer Register using ICD-10 diagnosis codes. Resource use in the health care sector was estimated for the year prior to diagnosis, and for the first, second and third years after diagnosis. Hospital resource use was defined in terms of registered hospital contacts, using DRG (Diagnosis Related Groups) and DAGS (Danish Outpatient Groups System) charges as cost estimates for inpatient and outpatient contacts, respectively. Health care consumption by cancer patients diagnosed in 2004-2007 was compared with that by an age- and sex-matched cohort without cancer. Hospital costs attributable to four anogenital cancers were estimated using regression analysis.
RESULTS: The annual incidence of anal cancer in Denmark is 1.9 per 100,000 persons. The corresponding incidence rates for penile, vaginal and vulvar cancer are 1.7, 0.9 and 3.6 per 100,000 males/females, respectively. The total number of new cases of these four cancers in Denmark is about 270 per year. In comparison, the total number of new cases cervical cancer is around 390 per year. The total cost of anogenital cancer to the hospital sector was estimated to be 7.6 million Euros per year. Costs associated with anal and vulvar cancer constituted the majority of the costs.
CONCLUSIONS: Anogenital cancer incurs considerable costs to the Danish hospital sector. It is expected that the current HPV vaccination program will markedly reduce this burden.


Monajemzadeh M, Vasei M, Kalantari M, et al.
Vulvar fibrous hamartoma of infancy: a rare case report and review of literature.
J Low Genit Tract Dis. 2013; 17(1):92-4 [PubMed]
BACKGROUND: Fibrous hamartoma of infancy is a benign mesenchymal tumor occurring infrequently in children, typically involving the axilla.
CASE REPORT: An 18-month-old girl with a history of right labium majus enlargement, on examination, had a hard mass that was found strictly adherent to subcutaneous tissue and overlying skin. Postexcision histological examination showed arranged spindle cells, adipose tissue, and nests of immature small cells in a myxoid background, consistent with fibrous hamartoma of infancy.
CONCLUSIONS: The main problem in the diagnosis is differentiating this lesion from soft tissue sarcomas, which require an aggressive therapeutic approach. Both surgeons and pathologists need to be aware of the existence of such benign condition in this unusual place to avoid unnecessary therapies.


VanSandt AM, Bronson J, Leclair C, et al.
Low-grade fibromyxoid sarcoma of the vulva: a case report.
J Low Genit Tract Dis. 2013; 17(1):79-81 [PubMed]
OBJECTIVE: The study aimed to describe a case of low-grade fibromyxoid sarcoma arising from the vulva and to discuss the diagnostic challenges, clinical management, and epidemiology of this rare malignancy.
CASE: A 36-year-old woman presented to 3 separate emergency departments with complaints of a painful and slowly enlarging vulvar mass. Eventual gynecologic referral resulted in excision of a 6-cm, noncystic vulvar mass. Pathological diagnosis revealed low-grade fibromyxoid sarcoma. Later, a right radical hemivulvectomy ensured adequate margins, and 2 years later, the patient is free of recurrent and metastatic disease.
CONCLUSIONS: Low-grade fibromyxoid sarcoma is a rare malignancy that may present in the lower genital tract. Definitive diagnosis is essential because low-grade fibromyxoid sarcoma may metastasize many years after diagnosis, thereby requiring indefinite clinical surveillance.


Huang CC, Sheu CY, Chen TY, Yang YC
Aggressive angiomyxoma: a small palpable vulvar lesion with a huge mass in the pelvis.
J Low Genit Tract Dis. 2013; 17(1):75-8 [PubMed]
Aggressive angiomyxoma (AAM) is a rare soft tissue tumor typically in the pelvis and perineum in women of reproductive age, which is easily misdiagnosed. We describe a woman with vulvar AAM, initially mismanaged as a Bartholin cyst. However, a huge pelvic mass is noted on the following imaging studies. The characteristics of AAM on computed tomography and magnetic resonance imaging have been specified in the literature, but we further point out the potential value of sonography in diagnosing AAM. Besides, excisional biopsy may cause tumor bleeding in a case of AAM.


Moraloğlu Ö, Güngör T, Ozyer S, et al.
Recurrent proliferating trichilemmal tumor of the vulva: a case report.
J Low Genit Tract Dis. 2013; 17(1):71-4 [PubMed]
Proliferating trichilemmal tumor (PTT) is a rare but morphologically distinct tumor that usually arises on the scalp of elderly women. It is composed of multiple cysts consisting of squamous epithelium with trichilemmal keratinization without granular layer interposition. Vulvar proliferating trichilemmal cyst is very rare, with, to the best of our knowledge, only 3 cases previously reported in the literature. We describe a 39-year-old woman with recurrent PTT on the left labium majus of the vulva, which had been excised from the same side 5 years before. She had a palpable nodule, approximately 2 cm in size, which was firm, mobile, and nontender; without erythema and ulceration; and covered by normal skin on the vulva. There was no inguinal lymphadenopathy. The lesion was removed by wide surgical excision; because of the tissue elasticity, primary closure was possible. The pathology result was reported as proliferating trichilemmal carcinoma with tumor-free margins. Although local recurrence after wide excision is rare, we recommend complete excision for treatment of PTT and long-term follow-up because of the possibility of recurrence.


Al-Ojaimi EH, Abdulla MM
Giant epidermoid inclusion cyst of the clitoris mimicking clitoromegaly.
J Low Genit Tract Dis. 2013; 17(1):58-60 [PubMed]
We describe a rare case of clitoromegaly due to a large clitoral cyst that occurred spontaneously without any declared previous female genital mutilation. The cyst was excised successfully with good cosmetic results.


Wessels R, de Bruin DM, Faber DJ, et al.
Optical coherence tomography in vulvar intraepithelial neoplasia.
J Biomed Opt. 2012; 17(11):116022 [PubMed]
Vulvar squamous cell carcinoma (VSCC) is a gynecological cancer with an incidence of two to three per 100,000 women. VSCC arises from vulvar intraepithelial neoplasia (VIN), which is diagnosed through painful punch biopsy. In this study, optical coherence tomography (OCT) is used to differentiate between normal and VIN tissue. We hypothesize that (a) epidermal layer thickness measured in OCT images is different in normal tissue and VIN, and (b) quantitative analysis of the attenuation coefficient (μoct) extracted from OCT data differentiates VIN from normal vulvar tissue. Twenty lesions from 16 patients are imaged with OCT. Directly after data acquisition, a biopsy is performed. Epidermal thickness is measured and values of μoct are extracted from 200 OCT scans of normal and VIN tissue. For both methods, statistical analysis is performed using Paired Mann-Whitney-test. Correlation between the two methods is tested using a Spearman-correlation test. Both epidermal layer thickness as well as the μoct are different between normal vulvar tissue and VIN lesions (p < 0.0001). Moreover, no correlation is found between the epidermal layer thickness and μoct. This study demonstrates that both the epidermal thickness and the attenuation coefficient of vulvar epithelial tissue containing VIN are different from that of normal vulvar tissue.


Forman D, de Martel C, Lacey CJ, et al.
Global burden of human papillomavirus and related diseases.
Vaccine. 2012; 30 Suppl 5:F12-23 [PubMed]
The worldwide prevalence of infection with human papillomavirus (HPV) in women without cervical abnormalities is 11-12% with higher rates in sub-Saharan Africa (24%), Eastern Europe (21%) and Latin America (16%). The two most prevalent types are HPV16 (3.2%) and HPV18 (1.4%). Prevalence increases in women with cervical pathology in proportion to the severity of the lesion reaching around 90% in women with grade 3 cervical intraepithelial neoplasia and invasive cancer. HPV infection has been identified as a definite human carcinogen for six types of cancer: cervix, penis, vulva, vagina, anus and oropharynx (including the base of the tongue and tonsils). Estimates of the incidence of these cancers for 2008 due to HPV infection have been calculated globally. Of the estimated 12.7 million cancers occurring in 2008, 610,000 (Population Attributable Fraction [PAF]=4.8%) could be attributed to HPV infection. The PAF varies substantially by geographic region and level of development, increasing to 6.9% in less developed regions of the world, 14.2% in sub-Saharan Africa and 15.5% in India, compared with 2.1% in more developed regions, 1.6% in Northern America and 1.2% in Australia/New Zealand. Cervical cancer, for which the PAF is estimated to be 100%, accounted for 530,000 (86.9%) of the HPV attributable cases with the other five cancer types accounting for the residual 80,000 cancers. Cervical cancer is the third most common female malignancy and shows a strong association with level of development, rates being at least four-fold higher in countries defined within the low ranking of the Human Development Index (HDI) compared with those in the very high category. Similar disparities are evident for 5-year survival-less than 20% in low HDI countries and more than 65% in very high countries. There are five-fold or greater differences in incidence between world regions. In those countries for which reliable temporal data are available, incidence rates appear to be consistently declining by approximately 2% per annum. There is, however, a lack of information from low HDI countries where screening is less likely to have been successfully implemented. Estimates of the projected incidence of cervical cancer in 2030, based solely on demographic factors, indicate a 2% increase in the global burden of cervical cancer, i.e., in balance with the current rate of decline. Due to the relative small numbers involved, it is difficult to discern temporal trends for the other cancers associated with HPV infection. Genital warts represent a sexually transmitted benign condition caused by HPV infection, especially HPV6 and HPV11. Reliable surveillance figures are difficult to obtain but data from developed countries indicate an annual incidence of 0.1 to 0.2% with a peak occurring at teenage and young adult ages. This article forms part of a special supplement entitled "Comprehensive Control of HPV Infections and Related Diseases" Vaccine Volume 30, Supplement 5, 2012.


Coleman RL, Ali S, Levenback CF, et al.
Is bilateral lymphadenectomy for midline squamous carcinoma of the vulva always necessary? An analysis from Gynecologic Oncology Group (GOG) 173.
Gynecol Oncol. 2013; 128(2):155-9 [PubMed] Article available free on PMC after 01/02/2014
OBJECTIVE: To determine which patients with near midline lesions may safely undergo unilateral groin dissection based on clinical exam and lymphoscintigraphy (LSG) results.
METHODS: Patients participating in GOG-173 underwent sentinel lymph node (SLN) localization with blue dye, and radiocolloid with optional LSG before definitive inguinal-femoral lymphadenectomy (LND). This analysis interrogates the reliability of LSG alone relative to primary tumor location in those patients who had an interpretable LSG and at least one SLN identified. Primary tumor location was categorized as lateral (>2cm from midline), midline, or lateral ambiguous (LA) if located within 2cm, but not involving the midline.
RESULTS: Two-hundred-thirty-four patients met eligibility criteria. Sixty-four had lateral lesions, and underwent unilateral LND. All patients with LA (N=65) and midline (N=105) tumors underwent bilateral LND. Bilateral drainage by LSG was identified in 14/64 (22%) patients with lateral tumors, 38/65 (58%) with LA tumors and in 73/105 (70%) with midline tumors. At mapping, no SLNs were found in contralateral groins among those patients with LA and midline tumors who had unilateral-only LSGs. However, in these patients groin metastases were found in 4/32 patients with midline tumors undergoing contralateral dissection; none were found in 27 patients with LA tumors.
CONCLUSION: The likelihood of detectable bilateral drainage using preoperative LSG decreases as a function of distance from midline. Patients with LA primaries and unilateral drainage on LSG may safely undergo unilateral SLN.


Zizi-Sermpetzoglou A, Savvaidou V, Fournogerakis S, et al.
Dermatofibrosarcoma protuberans of the mons pubis.
Eur J Gynaecol Oncol. 2012; 33(5):537-9 [PubMed]
Dermatofibrosarcoma protuberans (DFSP) is a rare fibrous tumor with intermediate malignant potential and in rare cases the vulva is involved. The most common clinical presentation is a firm plaque with surrounding red to blue discoloration, or less often with multiple small subcutaneous nodules. The authors present a case of a 66-year-old woman who came to the hospital complaining of longstanding painless nodules in the area of the mons pubis. On physical examination, a diffuse area of erythematous induration involving the mons pubis was recognized and within this area there were smaller nodules. The histological diagnosis was dermatofibrosarcoma protuberans. Microscopically DFSP has a storiform pattern of uniform cytologically bland spindle cells, with a characteristic honeycomb pattern of infiltration into the subcutaneous fat. Immunohistochemical staining demonstrates strong positivity for vimentin and CD34. The treatment has been through a wide local excision (WLE), although microscopic tumor projections beyond the central tumor nodule explain the tumors propensity for local recurrence.


Cooper JC, Hew KE, Audlin KM, et al.
Synchronous of breast and vulvar Paget's disease: a case report.
Eur J Gynaecol Oncol. 2012; 33(5):534-6 [PubMed]
BACKGROUND: Synchronous Paget's disease of breast and vulva is extremely rare and has only been reported in the literature in one other case.
CASE: A 58-year-old postmenopausal woman was found to have crusting, bleeding, and discharge from left nipple, as well as vulvar pruritis at the same time. Biopsy of breast lesion demonstrated Paget's disease with an underlying foci of ductal carcinoma in-situ that required total mastectomy of left breast with sentinel node biopsy and breast reconstruction. For vulvar symptoms, the patient was initially diagnosed with dermatitis and topical ointment was prescribed. However, her symptoms persisted for the next several months, and she underwent vulvar biopsy that demonstrated Paget's disease. She underwent partial vulvectomy. Multiple episodes of recurrent vulvar Paget's disease were noted in the postoperative course that medical therapy with Imiquimod and a second partial vulvectomy was performed.
CONCLUSION: Synchronous of breast and vulvar Paget's disease is presented. There was a delay in diagnosing vulvar Paget's disease in this experienced case. While coincidence of breast and vulvar Paget's disease is likely, ectopic mammary tissue in vulvar as well as secondary metastasis from a focal lesion of breast Paget's disease needs to be carefully evaluated whenever the patient complains of vulvar symptoms in the setting of breast Paget's disease.


Velemínský M
Extremely large vulvar fibroma in a 15-year-old girl.
Neuro Endocrinol Lett. 2012; 33(6):600-2 [PubMed]
BACKGROUND: Fibromas and fibromyomas belong to the most common solid vulvar tumors. Their cause remains unknown.
CASE: A 15-year-old girl arrived at our department for extirpation of a large pendulous vulval fibroma. For three years she had observed a gradually enlarging structure protruding from her external genitals. After a preoperative CT examination the tumor was extirpated with a histological diagnosis of benign soft fibroma.
CONCLUSION: Our report describes a therapeutic management of a large vulval fibroma in a young girl. The extended time from first symptoms to final treatment deserves reflection.


Longpre MJ, Lange PH, Kwon JS, Black PC
Penile carcinoma: lessons learned from vulvar carcinoma.
J Urol. 2013; 189(1):17-24 [PubMed]
PURPOSE: Penile carcinoma is rare in the developed world and treatment guidelines are often based on marginal clinical data. Prospective controlled studies are virtually absent and meta-analyses are rare. Vulvar carcinoma, on the other hand, has many parallels to penile carcinoma, and the level of evidence for diagnosis and treatment is more robust. Therefore, we assessed the body of literature on vulvar carcinoma to identify potential improvements in the care of patients with penile carcinoma.
MATERIALS AND METHODS: A literature review was performed on vulvar carcinoma and direct comparisons were made to a similar review of the literature on penile carcinoma.
RESULTS: Several aspects of vulvar carcinoma management are clearly established and deserve closer evaluation in penile carcinoma. For example, human papillomavirus is identified in a high percentage of patients with vulvar carcinoma but is understudied in penile carcinoma. Further study is of potential clinical value, especially with the development of human papillomavirus vaccines for prevention. Penile carcinoma TNM staging does not adequately stratify survival or risk of advanced disease. Staging of vulvar carcinoma is dependent on tumor size and depth of invasion measured in millimeters, as opposed to the invasion of underlying structures in penile carcinoma. Management of the inguinal nodes is more refined for vulvar carcinoma, where lymphatic mapping has been conducted and sentinel node biopsy has proven to be highly effective in multicenter trials. Finally, the efficacy of adjuvant radiation and chemotherapy has been tested in controlled trials or reported in meta-analyses for vulvar carcinoma, which are both lacking for penile carcinoma. Radiation after inguinal node dissection, for example, has been shown to enhance survival in patients with defined risk factors. Neoadjuvant chemoradiation is recommended before surgery for advanced vulvar carcinoma.
CONCLUSIONS: Evidence derived from studies on vulvar carcinoma can be extrapolated to penile carcinoma to help guide clinical trials and future research directions to enhance the treatment of these patients.


Cinotti E, Perrot JL, Labeille B, et al.
Reflectance confocal microscopy for the diagnosis of vulvar melanoma and melanosis: preliminary results.
Dermatol Surg. 2012; 38(12):1962-7 [PubMed]
BACKGROUND: In the early stages, vulvar melanoma can mimic vulvar melanosis and therefore the diagnosis is often late and carries a poor prognosis. In vivo reflectance-mode confocal microscopy (RCM) is an emerging technique that allows noninvasive high-resolution imaging of the skin and mucosa, but it has not been employed in the study of genital pigmentation.
OBJECTIVE: To analyze the characteristics of vulvar melanosis and vulvar melanoma using RCM to define the confocal aspects that allow a correct differential diagnosis.
METHODS AND MATERIALS: Features of eight melanoses and two melanomas of the vulva were analyzed using RCM. RCM diagnosis was then compared with clinical and histologic diagnosis.
RESULTS: Two major characteristics are associated with vulvar melanosis: papillae rimmed by bright monomorphous cells and possible presence of a few dendritic bright cells in the basal layer of the epithelium. Two major features of vulvar melanoma have been identified: atypical cells in the epithelium and loss of normal architecture of chorion papillae.
CONCLUSIONS: Reflectance Confocal Microscopy can play a role in noninvasive differentiation between vulvar melanoma and vulvar melanosis, but further broader studies are needed to validate our observations.


Senn B, Mueller MD, Hasenburg A, et al.
Development of a postsurgical patient-reported outcome instrument for women with vulvar neoplasia.
Oncol Nurs Forum. 2012; 39(6):E489-98 [PubMed]
PURPOSE/OBJECTIVES: To (a) develop the Women With Vulvar Neoplasia-Patient-Reported Outcome (WOMAN-PRO) instrument as a measure of women's post-vulvar surgery symptom experience and informational needs, (b) examine its content validity, (c) describe modifications based on pilot testing, and (d) examine the content validity of the revised instrument.
DESIGN: Mixed-methods research project.
SETTING: One Swiss and two German university hospitals.
SAMPLE: 10 patients and 6 clinicians participated in the pilot test.
METHODS: The instrument was developed based on literature searches, clinician feedback, and patient interviews. Thirty-seven items were first pilot tested by patients and clinicians. The revised 36 items were pilot tested by patients. The content validity index (CVI) for each item and the entire instrument was calculated.
MAIN RESEARCH VARIABLES: Symptom experience and informational needs of patients with vulvar neoplasia.
FINDINGS: The initial pilot test showed excellent scale CVI based on feedback from patients (CVI = 0.98) and clinicians (CVI = 0.92). After revising six items based on low individual CVIs and participant comments, the revised WOMAN-PRO showed excellent item and scale content validity (CVI = 1).
CONCLUSIONS: The newly developed WOMAN-PRO instrument can guide patients and clinicians in assessing symptoms, informational needs, and related distress.
IMPLICATIONS FOR NURSING: Use of the WOMAN-PRO instrument in clinical practice can offer patients guidance in self-assessment and early recognition of symptoms. The instrument also can provide clinicians with systematic information about key symptoms from a patient perspective, as well as women's unmet informational needs. If the results of additional psychometric testing are promising, the WOMAN-PRO tool may provide an outcome measure for clinical trials.


Wellenhofer A, Brustmann H
Expression of human telomerase reverse transcriptase in vulvar intraepithelial neoplasia and squamous cell carcinoma: an immunohistochemical study with survivin and p53.
Arch Pathol Lab Med. 2012; 136(11):1359-65 [PubMed]
CONTEXT: Human telomerase reverse transcriptase (hTERT), an enzyme that enables cells to overcome replicative senescence and to divide indefinitely, is overexpressed in many cancers and their precursor lesions.
OBJECTIVE: To test whether hTERT expression is related to neoplastic progression and resistance to apoptosis in vulvar epithelia.
DESIGN: Immunoexpression of hTERT was evaluated in 101 formalin-fixed, paraffin-embedded archival vulvar epithelia consisting of normal squamous vulvar epithelia (n  =  25), lichen sclerosus (n  =  10), high-grade classic vulvar intraepithelial neoplasia (n  =  16), differentiated vulvar intraepithelial neoplasia (n  =  18), and vulvar invasive keratinizing squamous cell carcinoma (n  =  32) and related to survivin and p53 expression. Immunostaining for all factors was scored for moderate and strong intensities with regard to quantity to determine upregulation and overexpression (score 0, 0% immunoreactive cells; score 1+, <5% immunoreactive cells; score 2+, 5% to 50% immunoreactive cells; score 3+, >50% immunoreactive cells). Score 3+ was considered as overexpression.
RESULTS: Nuclear hTERT immunoexpression was closely related to survivin reactivity, increased from normal vulvar squamous epithelia to lichen sclerosus and to high-grade classic vulvar intraepithelial neoplasia, differentiated vulvar intraepithelial neoplasia, and invasive keratinizing squamous cell carcinoma (P < .001), and followed the morphologic distribution of atypical squamous epithelial cells. Overexpression of hTERT was comparable to that seen for p53 in invasive keratinizing squamous cell carcinoma (P  =  .62); significant differences were calculated for differentiated vulvar intraepithelial neoplasia (P  =  .003) and high-grade classic vulvar intraepithelial neoplasia (P  =  .001).
CONCLUSION: Human telomerase reverse transcriptase is upregulated in vulvar intraepithelial neoplasia and invasive keratinizing squamous cell carcinoma compared with nonneoplastic squamous epithelia of the vulva as an apparently early and preinvasive event in the neoplastic transformation, with development of cellular longevity and resistance to apoptosis by survivin activation as associated features, independent of the etiology of vulvar intraepithelial neoplasia.


Gunia S, Koch S, May M
Is CDX2 immunostaining useful for delineating anorectal from penile/vulvar squamous cancer in the setting of squamous cell carcinoma with clinically unknown primary site presenting with histologically confirmed inguinal lymph node metastasis?
J Clin Pathol. 2013; 66(2):109-12 [PubMed]
AIMS: Penile, vulvar and anal squamous cell carcinomas (SCCs) share histomorphological overlap and are prone to lymphatic dissemination into inguinal nodes. Anal SCCs might derive from the anorectal zone (ARZ), anal transitional zone, squamous zone or from perianal skin. These anatomically distinct zones differ in terms of their embryological development. We sought to investigate the role of caudal-related homeobox 2 (CDX2), a homeobox gene implicated in the development and anterior/posterior pattern specification from duodenum to rectum including the ARZ, in terms of narrowing the possible sites of origin to be considered in the setting of SCC with unknown primary presenting with histologically confirmed inguinal lymph node metastasis.
METHODS: By immunohistochemistry (IHC) employing a panel of antibodies directed against CK5/6, CK7, CK20, p63, p16, CEA and CDX2, we compared 89 penile, 11 vulvar and eight anal SCCs with respect to their staining profiles. Moreover, anal SCCs were subjected to in situ hybridisation (ISH) for high-risk human papillomavirus (HPV) subtypes.
RESULTS: By IHC, CDX2 expression was observed in 2/8 anal SCCs (25%) while being absent from all penile and vulvar SCCs examined. High-risk HPV subtypes were detected by ISH in all anal SCCs examined, which were uniformly p16-positive by IHC.
CONCLUSIONS: CDX2 might be valuable in terms of narrowing the possible sites of origin to be considered in the setting of SCC with unknown primary presenting with inguinal lymph node metastasis. However, despite its favourable specificity, the diagnostic benefit achieved by this observation is limited by the low sensitivity.


Li YZ, Li SL, Li X, et al.
Expression of endogenous hypoxia markers in vulvar squamous cell carcinoma.
Asian Pac J Cancer Prev. 2012; 13(8):3675-80 [PubMed]
OBJECTIVE: To investigate the expression of endogenous hypoxia-related markers identified as being involved in vulvar squamous cell carcinoma (VSCC).
METHODS: We performed immunohistochemical staining of hypoxia-inducible factor-1α(HIF-1α), glucose transporter-1 (GLUT-1), carbonic anhydrase 9 (CA-9) and vascular endothelial growth factor (VEGF), on tissue sections of 25 VSCC patients, 10 vulvar intraepithelial neoplasia (VIN) patients and 12 healthy controls.
RESULTS: HIF-1α expression was found in all sections, with no significant difference between controls, VIN and VSCC sections (all P<0.05). Glut-1 expression was found in 25% of control, 90% of VIN and 100% of VSCC sections. A significant difference between control and VIN or VSCC was observed (all P<0.05), while no difference was found between VIN and VSCC sections (P>0.05). CA-9 expression was negative in control sections, but it was found in 30% of VIN sections and 52% of VSCC sections with strong staining. Similarly, CA-9 expression also showed obvious differences between controls and VIN or VSCC sections (all P<0.05). However, there was no significant difference between VIN and VSCC (P>0.05). There were only 25% of control sections with weak VEGF expression, while strong staining was found in about 60% of VIN sections and 25% of VSCC sections (all P<0.05). In addition, a difference was also found between VIN and VSCC sections (P<0.05).
CONCLUSION: Expression of endogenous hypoxia markers (HIF-1α, GLUT-1, CA-9 and VEGF) might be involved in the malignant progression of VSCC.


Messalli EM, D'Aponte ML, Luise R, et al.
An apparently benign vulvar mass: possibly a rare malignancy.
Eur J Gynaecol Oncol. 2012; 33(4):441-4 [PubMed]
BACKGROUND: Vulvar dermatofibrosarcoma is a rare fibrous tumor of intermediate grade malignancy, with a tendency for local recurrence, and rarely metastasizes. Management should be multidisciplinary. This is a report of an apparently benign vulvar mass with delayed diagnosis of vulvar dermatofibrosarcoma.
CASE REPORT: A 42-year-old woman was referred to our hospital because of a vulvar tumor lasting 16 years, although several gynecological procedures and a total laparoscopic hysterectomy had been performed two years before. During this long period the lesion did not change morphological features and remained asymptomatic. Only a benign vulvar mass was diagnosed. Then, the swelling became evident showing erythematous skin with an aspect of "peau d'orange", leading the patient to consult a specialist. A firm vulvar swelling was observed in the anterior third of right labia majora continuing with about 3 cm of cord on top, quite movable above the underlying tissue but not on the overlying tissue. A wide excision was performed. The pathological examination showed positive margins. One month later an extensive deeper excision was performed. Histology confirmed a diagnosis of dermatofibrosarcoma. Immunohistochemistry was strongly positive for CD34.
CONCLUSION: Vulvar lesions always require complete pathologic examination even in case of features of benign tumor to exclude a dermatofibrosarcoma. The role of the pathologist is essential to ensure negative microscopic margins and to avoid local recurrence.


Cormio G, Carriero C, Loizzi V, et al.
"Intestinal-type" mucinous adenocarcinoma of the vulva: a report of two cases.
Eur J Gynaecol Oncol. 2012; 33(4):433-5 [PubMed]
BACKGROUND: "Intestinal-type" mucinous carcinoma of the vulva is extremely rare with very few cases reported in the literature.
CASE REPORT: The authors report two patients who had diagnosis of intestinal-type mucinous adenocarcinoma of the vulva after excisional biopsy. In both cases, restaging was perfomed with total body computed tomography (CT) scan, gastroscopy, and colonoscopy that showed no other site of disease. A radical vulvectomy with bilateral systematic inguinal lymphadenectomy was performed, and in both cases no residual disease was found. A patient developed metastatic (liver, bone marrow) colonic cancer 36 months after primary surgery, received multiple lines of chemotherapy, and died of disseminated disease 18 months after diagnosis. The other patient was found to have dysplastic polyp in the sigmoid colon, and is alive without disease at 39 months after primary diagnosis.
CONCLUSION: Intestinal-type mucinous carcinoma of the vulva has a poor prognosis. Strict endoscopic follow-up of the colon is mandatory in such cases, considering the high propensity of associated gastrointestinal (GI) tumors.


Santeufemia DA, Capobianco G, Re GL, et al.
Cisplatin-gemcitabine as palliative chemotherapy in advanced squamous vulvar carcinoma: report of two cases.
Eur J Gynaecol Oncol. 2012; 33(4):421-2 [PubMed]
Vulvar cancer (VC) is a rare disease, usually diagnosed in a stage still amenable to potentially curative treatments, including surgery and/or radiation therapy with or without chemotherapy. Several patients however present at diagnosis with metastatic disease and another 30-50% will relapse. Prognosis of metastatic or recurrent disease not amenable to salvage surgery or radiotherapy is very poor. Evidence about the efficacy of chemotherapy in this setting is limited and its role still remains unclear. At present there is no standard treatment for advanced VC and patients are usually treated with schedules adopted for chemoradiation or extrapolated from cervical cancer. We report our experience using a cisplatin-gemcitabine regimen in two cases of metastatic squamous cell VC. No response was obtained with this schedule. No other data are available in the literature about the choice of a cisplatin-gemcitabine regimen in this patient subset. The paucity of evidence about the role of palliative chemotherapy in metastatic VC justifies any effort to implement knowledge. For this reason we think it is notable to also report a negative experience. It is not possible for us to conclude that this chemotherapy would be unable to provide any benefit in a larger sample of patients; nonetheless we think that new agents, rather than combinations of older drugs, could hopefully provide more benefit.


Tolia M, Tsoukalas N, Platoni K, et al.
Metastatic bone involvement in vulvar cancer: report of a rare case and review of the literature.
Eur J Gynaecol Oncol. 2012; 33(4):411-3 [PubMed]
PURPOSE: Bone metastasis secondary to vulvar carcinoma is an infrequent clinical entity. Only ten cases have been published in the literature. We describe a case of squamous vulvar carcinoma, that presented with cervical vertebral involvement, as a part of distant spread.
CASE: A 69-year-old woman presented with radicular pain and a painful cervical mass. MRI of the cervical spine was performed, showing an osteolytic lesion with spinal cord compression.
CONCLUSION: This case was unique in presenting vertebral metastasis eight months after chemotherapy and radiotherapy.


Fallani MG, Fambrini M, Lozza V, et al.
CO2 laser total superficial vulvectomy: an outpatient treatment for wide multifocal vulvar intraepithelial neoplasia grade 3.
J Minim Invasive Gynecol. 2012 Nov-Dec; 19(6):758-61 [PubMed]
The ideal treatment of large multifocal vulvar intraepithelial neoplasia grade 3 (VIN 3) in young patients is still debated. The goal is to prevent development of invasive vulvar cancer while preserving normal vulvar anatomy and function. The authors describe the case of a 37-year-old woman affected by a biopsy-proven VIN 3 involving the entire external genitalia. A total superficial vulvectomy was carried out in 2 closer sessions by CO(2) laser used in an excisional way. Both procedures were performed in an outpatient setting with the patient under local anesthesia and without suturing stitches or skin flaps. Definitive pathologic analysis confirmed VIN 3 with free margins. No intraoperative and postoperative complications were documented. Functional and anatomic outcomes were optimal, and no relapse occurred after 12 months of follow-up. Use of CO(2) laser total superficial vulvectomy shows promise of a safe and adequate treatment in selected young patients with VIN 3 involving the entire external genitalia.


Rumbold AR, Tan SE, Condon JR, et al.
Investigating a cluster of vulvar cancer in young women: a cross-sectional study of genital human papillomavirus prevalence.
BMC Infect Dis. 2012; 12:243 [PubMed] Article available free on PMC after 01/02/2014
BACKGROUND: Vulvar cancer is a relatively rare malignancy, which occurs most often in postmenopausal women. We have previously identified a geographic cluster of vulvar cancer in young Indigenous women living in remote communities in the Arnhem Land region of Australia. In this population, we investigated the prevalence of oncogenic human papillomavirus (HPV) infection in anogenital samples (vulvar/vaginal/perianal area and cervix) and compared the overall, type-specific and multiple infection prevalence between sites.
METHODS: A cross-sectional survey of 551 Indigenous women aged 18-60 years was undertaken in 9 Arnhem Land communities. Women were consented for HPV detection and genotyping collected by a combined vulvar/vaginal/perianal (VVP) sweep swab and a separate PreservCyt endocervical sample collected during Pap cytology screening. HPV DNA testing was undertaken using PCR with broad spectrum L1 consensus PGMY09/11 primers with genotyping of positive samples by Roche Linear Array. The primary outcomes were the prevalence of cervical and VVP high-risk (HR) HPV.
RESULTS: The prevalence of VVP HR-HPV was 39%, which was significantly higher than the cervical HR-HPV prevalence (26%, p<0.0001). HPV-16 was the most common genotype detected in both sites (VVP 11%, cervical 6%). HPV-16 infection peaked in women aged <20 years; however, there was a marked decline in cervical HPV-16 prevalence with age (p=0.007), whereas following an initial decline, the prevalence of VVP HPV-16 remained constant in subsequent age-groups (p=0.835).
CONCLUSIONS: In this population experiencing a cluster of vulvar cancer, the prevalence of cervical oncogenic HPV infection was similar to that reported by studies of other Australian women; however there was a significantly higher prevalence of vulvar/vaginal/perianal infection to cervical. The large discrepancy in HPV prevalence between anogenital sites in this population may represent more persistent infection at the vulva. This needs further investigation, including the presence of possible environmental and/or genetic factors that may impair host immunity.


This page last updated: 22nd May 2013
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