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Vulva Cancer

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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  • PubMed search for publications about Vulvar Cancer - Limit search to: [Reviews]

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    MeSH term: Vulvar Neoplasms
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Latest Research Publications

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

Ammouri S, Elkarkri C, Zeraidi N, et al.
Vulvar leiomyoma: a case report.
Pan Afr Med J. 2019; 32:208 [PubMed] Free Access to Full Article Related Publications
Leiomyomas represent about 3.8% of all benign soft tissue tumors. Vulvar localization is very rare. We present a case of a vulvar leiomyoma and discuss diagnostic and therapeutic features of this disease. A 30-year-old female patient with no medical history, had a 5cm mass located in the left large lip causing a discomfort at the perineum especially in sitting and walking. She underwent a complete surgical excision of the mass. The pathological examination confirmed the diagnosis of a leiomyoma. There was no recurrence after 24-months' follow-up. The vulvar leiomyoma is a rare benign tumor. The diagnosis is made only postoperatively after resection of the mass. The treatment is essentially based on total excision of the mass with a good prognosis.

Garganese G, Inzani F, Mantovani G, et al.
The vulvar immunohistochemical panel (VIP) project: molecular profiles of vulvar Paget's disease.
J Cancer Res Clin Oncol. 2019; 145(9):2211-2225 [PubMed] Related Publications
PURPOSE: To investigate the expression of biological markers in primary vulvar Paget's disease (VPD).
METHODS: Forty-one patients referred to a single major Center for Gynecologic Oncology from January 2008 to June 2018 were enrolled retrospectively: 30 non-invasive-VPD and 11 invasive-VPD. A total number of 60 samples, from all the 41 vulvar sites (VS), 8 metastatic lymph node sites (MLS) and 11 successive recurrent disease in vulvar site (RVS), were tested for an immunohistochemical panel, including the following markers: PD-L1, CD3, MSH2, MSH6, MLH1, PMS2, HER2/neu, EGFR, p16, p53, Ki67, ER, PR, AR, VEGF and CD31.
RESULTS: We found a positive PD-L1 in 10% of non-invasive-VPD and 27% of invasive-VPD (18% VS; 38% MLS). ER and AR were expressed respectively in more than 70% and 75% of all specimens. HER2/neu amplification was found in 21% of non-invasive-VPD and 45% of invasive-VPD (40% VS; 38% MLS). A machine learning cluster analysis identified three groups among non- invasive-VPD: cluster-1 with higher median ER expression (40%); cluster-3 with more frequent HER2/neu overexpression (46%). Among invasive-VPD, two clusters were found: the second with more frequent HER2/neu overexpression (67% vs. 0%) and nodal metastases (100% vs. 25%). Repeating the IHC panel on the correspondent MLS and RVS, it significantly changed, respectively, in 50% and 27%.
CONCLUSIONS: This study reveals the expression of PDL-1 and ER and confirms the expression of HER2/AR in VPD; new bases are provided to design multicenter clinical trials on personalized target therapies.

Sánchez-García A, García Moreno MÁ, Salmerón-González E, et al.
Inguinal Reconstruction Using Pedicled Rectus Abdominis Flap: A Useful Option for the Application of Radiotherapy.
Plast Surg Nurs. 2019 Apr/Jun; 39(2):41-43 [PubMed] Related Publications
Given their high rate of complications, radical surgical procedures of anorectal and gynecological tumors require a reliable and individualized reconstruction. The latter is influenced by the frequent indication of adjuvant chemo/radiotherapy that they present. We describe the case of a patient with medical history of vulvar carcinoma that required radical surgery and bilateral inguinal lymphadenectomy. Because of the stage of the tumor, the application of postoperative radiotherapy was clinically indicated; however, after surgery, the patient developed bilateral inguinal ulcers that made postoperative radiotherapy application impossible. Using a radical surgical approach in combination with postoperative radiotherapy increases survival in patients with these types of tumors. Therefore, delaying its use because of wound complications or inadequate reconstruction cannot be justified. The pedicled abdominal rectus flap is an excellent option for this purpose in patients with moderate- to large-sized defects.

Guglielmo P, Paderno M, Elisei F, et al.
18F-FDG PET/CT in a Case of Metastatic Breast Cancer to the Vulva.
Clin Nucl Med. 2019; 44(7):572-573 [PubMed] Related Publications
A 56-year-old woman, previously treated for breast cancer, presented to gynecologists with a lump of the left labium major of the vulva. The FDG pattern resembled as a cIV stage vulvar cancer, whereas biopsy indicated metastases from breast cancer. Metastatic disease to the vulva is particularly uncommon, representing 5% to 8% of all vulvar cancer lesions. However, gynecological uptakes, including vulvar site, have to be kept in mind as a possible site of metastatic lesions from breast cancer.

Thangarajah F, Morgenstern B, Pahmeyer C, et al.
Clinical impact of PD-L1 and PD-1 expression in squamous cell cancer of the vulva.
J Cancer Res Clin Oncol. 2019; 145(6):1651-1660 [PubMed] Related Publications
PURPOSE: Squamous cell carcinoma of the vulva (SQCV) is the fifth most common cancer in women and accounts for about 5% of all genital cancers in women. The PD-L1 signaling pathway is activated in many malignant neoplasms and its blockade enhances anti-cancer immunity. The aim of our study was to examine the protein expression of PD-L1 and PD-1 in squamous cell cancer of the vulva, its correlations with clinicopathologic features and prognostic value.
METHODS: Patients with SQCV treated in one institution were used for the analyses. PD-L1 immunohistochemistry was performed on 4 µm-thick section of the respective FFPE tissue blocks using the 28-8 antibody. PD-L1 scoring was performed separately for tumour cells (TC) and tumour associated immune cells. DNA was extracted to determine HPV status. Kaplan-Meier estimates for disease-free-survival and overall-survival were calculated and compared by log-rank test.
RESULTS: PD-L1 expression in tumour cells could be observed in 32.9% of the patients. The expression of PD-L1 in peritumoural immune cells was confirmed in 91.4% of the patients. A significant correlation between PD-L1 expression in tumour cells and tumour stage was detected (p = 0.007). PD-L1 expression was independent from HPV status. Using the log-rank test we could not prove any significant differences in disease-free survival (p = 0.434) and overall survival (p = 0.858). Regression analysis showed that nodal status is a predictive factor of survival (p < 0.001).
CONCLUSION: The present study showed that a relevant amount of patients with squamous cell cancer of the vulva express PD-L1 in both, tumour cells and tumour-associated immune cells. Furthermore, the significant correlation of PD-L1 expression in TCs with tumour stage indicated the clinical impact of PD-L1 expression during tumour development. These data indicate that SQCV might be amenable to immune checkpoint-inhibition and constitute a rational for the future clinical trials.

Wiggans A, Coleridge S, Bryant A, Morrison J
Relationship between vulvar symptoms and incidence of vulvar cancer in women referred to a rapid access clinic.
Int J Gynaecol Obstet. 2019; 145(3):283-286 [PubMed] Related Publications
OBJECTIVE: We performed a study to estimate incidence of vulvar cancer in women with vulvar symptoms (irritation, pain, bleeding +/- presence of lesion) referred to a secondary care, rapid-access clinic.
METHODS: Prospective data collection of all direct referrals from a primary to a secondary care gynecological oncology clinic from 2011 to 2016, for women with suspicious vulvar symptoms.
RESULTS: 32/393 (8.1%) women had vulvar cancer, and 24/393 (6.1%) had a premalignant lesion. Multivariate logistic regression showed that women referred without a specific lesion had considerably lower odds of a diagnosis of vulvar cancer than those with a lesion (OR=0.11, 95% CI: 0.03-0.49). In total, 30/234 (12.8%) women with a vulvar lesion (mass or ulcer), had vulvar cancer, compared with 2/159 (1.3%) of those referred without a lesion (these patients had vulvar irritation and bleeding but had a visible lesion on examination). None of the 140 women with irritation alone, in the absence of a visible lesion or bleeding, had pre-invasive disease or cancer.
CONCLUSION: Presence of a vulvar lesion, especially if painful/bleeding, has a high positive predictive value for vulvar cancer and 12.8% of women presenting with any vulvar lesion to secondary care had cancer.

Fu S, Fokom Domgue J, Chan W, et al.
Cervical, Vaginal, and Vulvar Cancer Costs Incurred by the Medicaid Program in Publicly Insured Patients in Texas.
J Low Genit Tract Dis. 2019; 23(2):102-109 [PubMed] Related Publications
OBJECTIVES: To determine from the perspective of the State of Texas, the direct medical care costs associated with cervical, vaginal, and vulvar cancers in Texas Medicaid enrollees.
MATERIALS AND METHODS: We conducted a case-control study and searched Texas Medicaid databases between 2008 and 2012 for eligible cancer patients. A comparison group was selected for each cancer site using a 2-step 1:1 propensity score matching method. Patients were followed for 2 years after cancer diagnosis to estimate monthly and yearly direct medical costs. For each cancer site, the differential cost between patients and the matched comparison individuals was the estimated cost associated with cancer.
RESULTS: The study included 583 cervical, 62 vaginal, and 137 vulvar cancer patients and equal numbers of cancer-free comparison individuals. Among the cases, 322 cervical cancer patients, 46 vaginal cancer patients, and 102 vulvar cancer patients were Medicaid-Medicare dual eligible enrollees. For Medicaid-only enrollees, the adjusted first- and second-year mean total differential costs were US $19,859 and $3,110 for cervical cancer, US $19,627 and $4,582 for vaginal cancer, and US $7,631 and $777 for vulvar cancer patients, respectively. For Medicaid-Medicare dual eligible enrollees, adjusted first- and second-year mean total differential costs incurred by Medicaid were US $2,565 and $792 for cervical cancer, US $1,293 and $181 for vaginal cancer, and US $1,774 and $1,049 for vulvar cancer patients, respectively.
CONCLUSIONS: The direct medical costs associated with cervical, vaginal, and vulvar cancers in Texas Medicaid were substantial in the first 2 years after cancer diagnosis, but dual eligibility for Medicare coverage attenuated Medicaid costs.

Zigras T, Kupets R, Barbera L, et al.
Uptake of sentinel lymph node procedures in women with vulvar cancer over time in a population based study.
Gynecol Oncol. 2019; 153(3):574-579 [PubMed] Related Publications
OBJECTIVES: To evaluate trends in uptake of sentinel lymph node (SLN) procedures over time and associated factors in women with vulvar cancer.
METHODS: A retrospective population-based cohort study identified women with invasive squamous cell carcinoma (SCC) of the vulva using health administrative data for the province of Ontario, Canada, between 2008 and 2016. Patients who underwent SLN procedures were compared to those who had groin node dissection (GND). Multivariable analysis was used to identify factors associated with SLN procedures.
RESULTS: 1385 patients with SCC of the vulva were identified; 1079 had a surgical procedure. Only those with groin node assessment were included in the study cohort (n = 732, 68%). SLN procedures were done in 52%. When comparing SLN versus GND, the rate of SLNs was significantly different by year of diagnosis (P < 0.001), associated comorbidity (P < 0.001) and institution (P < 0.0001). The rates of SLNs by institution with gynecologic oncologist were variable and ranged from 32% to 79% among 9 centers. There were no differences in age, income quintile, and urban/rural residence. The proportion of SLN procedures increased from 30.1% (CI 18.9-45.6) in 2008 to 65.2% (CI 36.5-107.6) in 2016. On multivariate analysis, factors significantly associated with SLN procedures were more recent year of diagnosis (OR 7.9, CI 2.7-23.5) associated comorbidities (OR 2.7, CI 1.5-5.0) and institution (Site 5, OR 19.6 [CI 3.6-108.3] and Site 6, [OR 6, CI 1.1-33.4]).
CONCLUSIONS: The proportion of SLN procedures in women with vulvar cancer has increased over time, but uptake is not uniform across institutions. Barriers to uptake should be explored.

Sykes P, Eva L, van der Griend R, et al.
Pathological process has a crucial role in sentinel node biopsy for vulvar cancer.
Gynecol Oncol. 2019; 153(2):292-296 [PubMed] Related Publications
OBJECTIVES: To report the interim findings of an audit of the outcomes of sentinel node (SN) biopsy performed as a replacement for groin node dissection in women with early stage vulvar cancer in routine clinical practice in Australia and New Zealand.
METHODS: A prospective multi-center study in 8 participating centers. Eligible patients had squamous cell carcinomas clinically restricted to the vulva <4 cm in diameter. SN procedures and pathological assessment were to be performed in accordance with the methods published by the GROINSS-V collaboration [1].
RESULTS: 130 women with apparent early stage vulvar cancer were enrolled. Seventeen women subsequently did not meet the eligibility criteria and were excluded. SNs were identified in 111/113 of the remaining women. Twenty-two women had positive nodes. Sixteen of these women had at least 12 months follow up and 7 (44%) had recurrent disease. Eighty-nine women had only negative nodes. Seventy-four of these women had at least 12 months follow up and 6 (8%) had recurrent disease (including 2 [2.7%] with recurrence in the groin). On subsequent review of the two women with negative SNs who had groin recurrences, it was found that the recommended pathology protocol had not been followed. In both cases, SN metastases were identified following serial sectioning of the nodes.
CONCLUSIONS: SN biopsy is feasible in routine clinical practice. However, undetected metastases in a removed SN may be associated with groin recurrence. To ensure patient safety, strict adherence to the pathology protocol is an essential component in the utilization of the sentinel lymph node technique in vulvar cancer.

Laliscia C, Gadducci A, Fabrini MG, et al.
Definitive radiotherapy for recurrent vulvar carcinoma after primary surgery: a two-institutional Italian experience.
Tumori. 2019; 105(3):225-230 [PubMed] Related Publications
OBJECTIVE: To assess the clinical outcome of patients treated with radiotherapy (RT) for recurrent squamous cell carcinoma of the vulva after primary surgery.
METHODS: Fifty-six patients developed recurrent disease after surgery, consisting of deep total vulvectomy with inguino-femoral lymphadenectomy in 44 (78.6%) and deep partial vulvectomy with inguino-femoral lymphadenectomy in 12 (21.4%). All patients underwent RT at the Divisions of Radiotherapy, University of Pisa and ASST Cremona, between 1992 and 2016. Forty-three patients (76.8%) underwent external beam RT and 13 (23.2%) were treated with exclusive high-dose rate brachytherapy.
RESULTS: Five-year progression-free survival (PFS) and overall survival (OS) were 19% and 43%, respectively. Primary tumor size ⩽4 cm, early FIGO stage, and negative lymph node status were significantly associated with better PFS (
CONCLUSIONS: Primary tumor size, FIGO stage, nodal status, and site of recurrent disease were significant predictors of clinical outcome in patients treated with RT for recurrent squamous cell carcinoma of the vulva.

Polterauer S, Schwameis R, Grimm C, et al.
Lymph node ratio in inguinal lymphadenectomy for squamous cell vulvar cancer: Results from the AGO-CaRE-1 study.
Gynecol Oncol. 2019; 153(2):286-291 [PubMed] Related Publications
OBJECTIVE: Lymph node ratio (LNR) can predict treatment outcome and prognosis in patients with solid tumors. Aim of the present analysis was to confirm the concept of using LNR for assessing outcome in patients with vulvar cancer after surgery with inguinal lymphadenectomy in a large multicenter project.
METHODS: The AGO-CaRE-1 study multicenter database was used for analysis. LNR was defined as ratio of number of positive lymph nodes (LN) to the number of resected. Previously established LNR risk groups were used to stratify patients. LNR was investigated with respect to clinical parameters. Univariate and multivariable survival analyses were performed to assess the value of LNR in order to predict overall (OS) and progression-free (PFS) survival.
RESULTS: In total, 1047 patients treated with surgery including inguinal lymph node resection for squamous cell carcinoma of the vulva were identified from the database. Of these, 370 (35.3%) were found to have positive inguinal LN. In total, 677 (64.7%) had a LNR of 0% (N0), 255 (24.4%) a LNR of >0% < 20%, and 115 (11%) a LNR of ≥20%. Patients with higher LNR were found to have larger tumor size (P < .001), advanced tumor stage (P < .001), high tumor grade (P < .001), and deep stromal invasion (P < .001), more frequently. Three-year PFS rates were 75.7%, 44.2%, and 23.1% and three-year OS rates were 89.7%, 65.4%, and 41.9%, in patients with LNRs 0%, >0% < 20%, and ≥20%, respectively (P < .001, P < .001). On multivariable analyses LNR (HR 7.75, 95%-CI 4.01-14.98, P < .001), FIGO stage (HR 1.41, 95%-CI 1.18-1.69, P < .001), and patient's performance status (HR 1.59, 95%-CI 1.39-1.82, P < .001), were associated with PFS. In addition, LNR (HR 12.74, 95%-CI 5.64-28.78, P < .001), and performance status (HR 1.72, 95%-CI 1.44-2.07, P < .001) were also the only two parameters independently associated with OS. LNR generally showed stronger correlation than number of affected LN when comparing the two different multivariable models.
CONCLUSIONS: In women with vulvar cancer LNR appears to be a consistent, independent prognostic parameter for both PFS and OS and allows patient stratification into three distinct risk groups. In survival analyses, LNR outperformed nodal status and number of positive nodes.

Corvino F, Giurazza F, Cangiano G, et al.
Endovascular Treatment of Peripheral Vascular Blowout Syndrome in End-Stage Malignancies.
Ann Vasc Surg. 2019; 58:382.e1-382.e5 [PubMed] Related Publications
BACKGROUND: Vascular blowout syndrome (VBOS) is a life-threatening condition secondary to direct tumor encasement or invasion in advanced stage malignancies. Endovascular management can be used as an alternative to surgical treatment in this fragile patient population, providing a minimally invasive measure both acutely and prophylactically.
METHODS: Three patients with peripheral VBOS secondary to advanced stage malignancies underwent successful endovascular treatment. Technical success was obtained in all patients with nonsignificant perioperative complications.
RESULTS: Endovascular management controlled immediate life-threatening hemorrhage and enabled these high-risk patients to undergo other adjunctive therapeutic modalities.
CONCLUSIONS: Endovascular treatment can offer a safe and effective palliative measure of peripheral VBOS secondary to neoplastic erosion in patients with advanced stage malignancies.

Boer FL, Ten Eikelder MLG, Kapiteijn EH, et al.
Vulvar malignant melanoma: Pathogenesis, clinical behaviour and management: Review of the literature.
Cancer Treat Rev. 2019; 73:91-103 [PubMed] Related Publications
Vulvar malignant melanoma (VMM) is a rare disease, accounting for 5% of all vulvar malignancies and is characterized by low survival and high recurrence rates. It is considered as a distinct entity of mucosal melanoma. Prognostic factors are higher age, advanced Breslow thickness, and lymph node involvement whilst central localization and ulceration status are still under debate. Surgery is the cornerstone for the treatment of primary VMM, however, it can be mutilating due to the anatomical location of the disease. Elective lymph node dissection is not part of standard care. The value of sentinel lymph node biopsy in VMM is still being studied. Radiation therapy and chemotherapy as adjuvant treatment do not benefit survival. Immunotherapy in cutaneous melanoma has shown promising results but clinical studies in VMM are scarce. In metastatic VMM, checkpoint inhibitors and in case of BRAF or KIT mutated metastatic VMM targeted therapy have shown clinical efficacy. In this review, we present an overview of clinical aspects, clinicopathological characteristics and its prognostic value and the latest view on (adjuvant) therapy and follow-up.

Weinberg D, Gomez-Martinez RA
Vulvar Cancer.
Obstet Gynecol Clin North Am. 2019; 46(1):125-135 [PubMed] Related Publications
This article reviews the epidemiology, diagnosis, and management of vulvar preinvasive lesions, squamous cell carcinoma, and melanoma. There is an emphasis on sentinel lymph node dissection for early stage disease and advances in chemoradiation for late-stage disease. A brief review of vulvar Paget disease is also included.

Ferrer Guillén B, Giácaman MM, Hernández Bel P, Pérez Ferriols A
Whitish vulvar tumors associated with macular symmetrical rash.
Dermatol Online J. 2018; 24(12) [PubMed] Related Publications
We present a woman with an unusual case of secondary syphilis after an unnoticed primary infection. She initially presented with multiple grayish plaques and nodules on the vulva associated with an erythematous macular symmetrical rash affecting the trunk and extremities. Despite the increasing incidence of sexually transmitted diseases such as syphilis, presentation with unusual manifestations can lead to a delayed diagnosis.

Ong KJ, Checchi M, Burns L, et al.
Systematic review and evidence synthesis of non-cervical human papillomavirus-related disease health system costs and quality of life estimates.
Sex Transm Infect. 2019; 95(1):28-35 [PubMed] Related Publications
BACKGROUND: Many economic evaluations of human papillomavirus vaccination should ideally consider multiple disease outcomes, including anogenital warts, respiratory papillomatosis and non-cervical cancers (eg, anal, oropharyngeal, penile, vulvar and vaginal cancers). However, published economic evaluations largely relied on estimates from single studies or informal rapid literature reviews.
METHODS: We conducted a systematic review of articles up to June 2016 to identify costs and utility estimates admissible for an economic evaluation from a single-payer healthcare provider's perspective. Meta-analyses were performed for studies that used same utility elicitation tools for similar diseases. Costs were adjusted to 2016/2017 US$.
RESULTS: Sixty-one papers (35 costs; 24 utilities; 2 costs and utilities) were selected from 10 742 initial records. Cost per case ranges were US$124-US$883 (anogenital warts), US$6912-US$52 579 (head and neck cancers), US$12 936-US$51 571 (anal cancer), US$17 524-34 258 (vaginal cancer), US$14 686-US$28 502 (vulvar cancer) and US$9975-US$27 629 (penile cancer). The total cost for 14 adult patients with recurrent respiratory papillomatosis was US$137 601 (one paper).Utility per warts episode ranged from 0.651 to 1 (12 papers, various utility elicitation methods), with pooled mean EQ-5D and EQ-VAS of 0.86 (95% CI 0.85 to 0.87) and 0.74 (95% CI 0.74 to 0.75), respectively. Fifteen papers reported utilities in head and neck cancers with range 0.29 (95% CI 0.0 to 0.76) to 0.94 (95% CI 0.3 to 1.0). Mean utility reported ranged from 0.5 (95% CI 0.4 to 0.61) to 0.65 (95% CI 0.45 to 0.75) (anal cancer), 0.59 (95% CI 0.54 to 0.64) (vaginal cancer), 0.65 (95% CI 0.60 to 0.70) (vulvar cancer) and 0.79 (95% CI 0.74 to 0.84) (penile cancer).
CONCLUSIONS: Differences in values reported from each paper reflect variations in cancer site, disease stages, study population, treatment modality/setting and utility elicitation methods used. As patient management changes over time, corresponding effects on both costs and utility need to be considered to ensure health economic assumptions are up-to-date and closely reflect the case mix of patients.

Linehan A, Harrold E, Pilson K, McCaffrey J
Recurrent vulvar melanoma in a patient with neurofibromatosis and gastrointestinal stromal tumour.
BMJ Case Rep. 2019; 12(1) [PubMed] Related Publications
We report a case of a 51-year-old woman with neurofibromatosis who presented in 2012 with postmenopausal bleeding. Excision biopsy of a pigmented lesion of the labia minora was consistent with an ulcerated vulvar BRAF wild type malignant melanoma (MM). Initial excision was followed by radical vulvectomy and adjuvant interferon. Local recurrence in January 2017 was further resected. Positron emission tomography (PET)-CT in May 2017 identified an FDG avid omental deposit; consistent histologically with MM when resected. Postoperative PET-CT in August 2017 demonstrated local recurrence. In the setting of resected stage IV disease and a third local recurrence, the decision was made to instigate immunotherapy. Vulvar melanoma is rare accounting for 0.2% of all melanoma. Presentation is typically a decade later than cutaneous melanoma with a tendency to late metastases and poorer prognosis. Given their rarity the treatment paradigm is less clearly defined and largely extrapolated from that of cutaneous melanomas.

Rottenstreich M, Armon S, Beller U, et al.
Recurrence of endometrial carcinoma presenting as vulvar lesions.
Int J Gynaecol Obstet. 2019; 145(1):123-124 [PubMed] Related Publications
Vulvar metastasis is an unusual presentation of recurrent endometrial cancer and should be part of the differential diagnosis of vulvar lesion.

Xie Y, Qian Y, Zou B
A giant aggressive angiomyxoma of vulva in a young woman: A case report.
Medicine (Baltimore). 2019; 98(2):e13860 [PubMed] Free Access to Full Article Related Publications
RATIONALE: Aggressive angiomyxoma (AAM) is a rare and benign invasive mesenchymal stromal tumor predominantly in women at reproductive age. AAM tends to relapse locally and should be differentially diagnosed from other mesenchymal tumors.
PATIENT CONCERNS: We report here a rare case of massive vulvar AAM in a 22-year-old Chinese woman with left labia majora mass with ulcer.
DIAGNOSES: The diagnosis "aggressive angiomyxoma of vulva" was based on clinicopathologic and immunohistochemical features.
INTERVENTIONS: A surgery with local excision of the mass was performed.
OUTCOMES: The patient was discharged 12 days after the surgery. There was no AAM recurrence or metastasis in a period of 12-month follow-up.
LESSONS: The vulvar AAM is a benign and aggressive mesenchymal tumor. In this case, we present the diagnosis, treatment, and prognosis for vulvar AAM. The tumor was removed completely by the surgery, but a long-term follow-up is requisite for surveilling on recurrence.

Bryan S, Barbara C, Thomas J, Olaitan A
HPV vaccine in the treatment of usual type vulval and vaginal intraepithelial neoplasia: a systematic review.
BMC Womens Health. 2019; 19(1):3 [PubMed] Free Access to Full Article Related Publications
BACKGROUND: HPV DNA is found in almost 80% of VIN/VaIN. Current management is inadequate, with high recurrence rates. Our objective was to review the literature regarding the role of HPV vaccine in secondary prevention and treatment of VIN/VaIN.
METHODS: Database searches included Ovid Medline, Embase, Web of Science, The Cochrane Library and Clinicaltrials.gov . Search terms included HPV vaccine AND therapeutic vaccine* AND VIN OR VAIN, published in English with no defined date limit. Searches were carried out with a UCL librarian in March 2018. We included any type of study design using any form of HPV vaccine in the treatment of women with a histologically confirmed diagnosis of VIN/VaIN. We excluded studies of other lower genital tract disease, vulval/vaginal carcinoma and prophylactic use of vaccines. The outcome measures were lesion response to vaccination, symptom improvement, immune response and HPV clearance.
RESULTS: We identified 93 articles, 7 studies met our inclusion criteria; these were uncontrolled case series. There were no RCTs or systematic reviews identified. Reduction in lesion size was reported by all 7 studies, symptom relief by 5, HPV clearance by 6, histological regression by 5, and immune response by 6.
CONCLUSIONS: This review finds the evidence relating to the use of HPV vaccine in the treatment of women with VIN/VaIN is of very low quality and insufficient to guide practice. Further longitudinal studies are needed to assess its use in prevention of progression to cancer.

Elumalai RK, Verma R, Malik D, Belho ES
Asymptomatic Isolated Vulvar Metastasis in Old Treated Case of Carcinoma Rectum-Diagnosis and Treatment Response Evaluation by 18F-FDG PET/CT Scan.
Clin Nucl Med. 2019; 44(3):e163-e165 [PubMed] Related Publications
We report a clinically asymptomatic, old treated case of carcinoma rectum, presenting with raised serum carcinoembryonic antigen level, referred for F-FDG PET/CT scan for restaging. Whole-body PET/CT scan revealed an isolated FDG-avid lesion in the left labia majora region, which on histopathology was found to be metastasis from carcinoma rectum. Subsequent F-FDG PET/CT done after 4 cycles of chemotherapy revealed favorable response to therapy. F-FDG PET/CT facilitates the detection of unusual site of metastasis and its subsequent response evaluation in the index case.

Xing D, Lu J
Distinctive clinicopathological features and disease‑specific survival of adenoid cystic carcinoma and adenoid basal carcinoma in the lower female genital tract.
Oncol Rep. 2019; 41(3):1769-1778 [PubMed] Related Publications
Adenoid cystic carcinomas (ACCs) and adenoid basal carcinomas (ABCs) in the lower female genital tract are very rare. Data on the clinicopathologic features and survival outcomes of ACCs and ABCs in the lower female genital tract are limited to case reports and small case series studies. The present study systemically analyzed 233 cases, including 84 cervical ACCs, 78 cervical ABCs and 71 vulvar ACCs, to identify clinicopathologic features and survival factors in a population‑based Surveillance, Epidemiology and End Results (SEER) study. Whereas cervical ACCs and ABCs tend to occur in the elderly (median, 72 and 69 years, respectively), vulvar ACCs commonly occurred in patients a decade younger (median, 59 years). The majority of patients with cervical ABC had localized disease and almost all received surgery. In contrast, cervical and vulvar ACC patients tended to have higher stage disease, and a significant proportion of these patients received radiotherapy, with or without surgery. The 5‑year cause‑specific survival (CSS) rates for patients with cervical ACC were 69.3%, vulvar ACC 87.7% and cervical ABC 96.6%. The 5‑year overall survival (OS) rate for patients with cervical ACC was 59.2%, significantly worse than that of cervical ABC (88.3%; P=0.002) and vulvar ACC (81.2%; P=0.01). Increased age and high stage were significantly associated with a worse prognosis in patients with cervical and vulvar ACCs by univariate and multivariate analysis (P<0.05). Tumor stage was the only significant factor associated with 5‑year overall survival in patients with cervical ABC (P<0.05). The present data demonstrated that the distinctive clinicopathologic features and survival outcomes differed significantly among ACCs and ABCs in the lower female genital tract, thus providing a rationale for location/pathologic type‑based treatment modalities.

Di Donato V, Page Z, Bracchi C, et al.
The age-adjusted Charlson comorbidity index as a predictor of survival in surgically treated vulvar cancer patients.
J Gynecol Oncol. 2019; 30(1):e6 [PubMed] Free Access to Full Article Related Publications
OBJECTIVE: To evaluate the impact of age-adjusted Charlson comorbidity index (ACCI) in predicting disease-free survival (DFS), overall survival (OS), and cancer-specific survival (CSS) among surgically treated patients with vulvar carcinoma. The secondary aim is to evaluate its impact as a predictor of the pattern of recurrence.
METHODS: We retrospectively evaluated data of patients that underwent surgical treatment for vulvar cancer from 1998 to 2016. ACCI at the time of primary surgery was evaluated and patients were classified as low (ACCI 0-1), intermediate (ACCI 2-3), and high risk (>3). DFS, OS and CSS were analyzed using the Kaplan-Meir and the Cox proportional hazard models. Logistic regression model was used to assess predictors of distant and local recurrence.
RESULTS: Seventy-eight patients were included in the study. Twelve were classified as low, 36 as intermediate, and 30 as high risk according to their ACCI. Using multivariate analysis, ACCI class was an independent predictor of worse DFS (hazard ratio [HR]=3.04; 95% confidence interval [CI]=1.54-5.99; p<0.001), OS (HR=5.25; 95% CI=1.63-16.89; p=0.005) and CSS (HR=3.79; 95% CI=1.13-12.78; p=0.03). Positive nodal status (odds ratio=8.46; 95% CI=2.13-33.58; p=0.002) was the only parameter correlated with distant recurrence at logistic regression.
CONCLUSION: ACCI could be a useful tool in predicting prognosis in surgically treated vulvar cancer patients. Prospective multicenter trials assessing the role of ACCI in vulvar cancer patients are warranted.

Nica A, Covens A, Vicus D, et al.
Sentinel lymph nodes in vulvar cancer: Management dilemmas in patients with positive nodes and larger tumors.
Gynecol Oncol. 2019; 152(1):94-100 [PubMed] Related Publications
BACKGROUND: Although sentinel lymph node (SLN) biopsy has been routinely used in the treatment of invasive squamous cell carcinoma (SCC), questions still remain regarding the management of patients with positive nodes, as well as its use in patients with larger tumors.
METHODS: Retrospective study of all patients at a single institution with primary vulvar cancer who had SLN biopsy (2008-2015). Patient and tumor characteristics were collected from hospital records. For patients with positive SLN and for those with tumors ≥40 mm, recurrence rates and location were specifically recorded.
RESULTS: SLN biopsy was successful in 159 patients (245 groins). Median follow-up was 31 months. 120 patients (187 groins) had a negative SLN without an inguinofemoral lymph node dissection (IFL); there were 6 ipsilateral groin recurrences (5%). 7 patients had micrometastasis (≤2 mm) in the SLN and were treated by radiotherapy. There were no recurrences in the irradiated groins. 19 patients with a positive unilateral SLN had bilateral IFL. One (5.3%) had a positive node in the contralateral groin. 9 patients with positive unilateral SLN had subsequent ipsilateral IFL; there were no groin recurrences in the contralateral groin. 20 patients had tumor size ≥40 mm. 11 patients had a negative SLN biopsy, and thus no IFL; of these patients, 1 had an isolated groin recurrence (9%).
CONCLUSION: These data suggest it is reasonable to omit a full groin dissection for micrometastatic disease in the SLN, and to perform a unilateral groin dissection in patients with unilateral SLN metastasis. SLN alone in larger tumors may have a higher groin recurrence rate.

Sand FL, Nielsen DMB, Frederiksen MH, et al.
The prognostic value of p16 and p53 expression for survival after vulvar cancer: A systematic review and meta-analysis.
Gynecol Oncol. 2019; 152(1):208-217 [PubMed] Related Publications
The tumor suppressor proteins p16 and p53 have been suggested to have prognostic value in some human papillomavirus (HPV)-associated cancers, however, this has been less well established for vulvar cancer. The aim of this review and meta-analysis was to examine the prognostic value of p16 and p53 expression status on survival after vulvar squamous cell carcinoma (VSCC). We conducted a thorough systematic literature search of multiple databases to identify studies examining survival after histolocally verified VSCC that were tested for p16 and/or p53. A total of 18 eligible studies were included. Using a fixed-effects model we calculated study-specific and pooled hazard ratios (HRs) of 5-year overall survival (OS). In the analyses of OS, we included 475 VSCC cases tested for p16 expression of which 38% were p16 positive. The pooled HR

Salcedo MP, Sood AK, Dos Reis R, et al.
Perineural invasion (PNI) in vulvar carcinoma: A review of 421 cases.
Gynecol Oncol. 2019; 152(1):101-105 [PubMed] Related Publications
OBJECTIVES: To evaluate the prevalence and associated prognostic indicators in patients with vulvar carcinoma with and without evidence of perineural invasion (PNI).
METHODS: A retrospective review identified 421 patients with invasive vulvar carcinoma evaluated at a single institution between 1993 and 2011. Medical records were reviewed for demographic data, pathologic information and presence or absence of PNI, treatment type, and recurrence/outcome information. Variables were compared between patients with PNI to those without PNI.
RESULTS: Of the 421 patients included in the study, 32 (7.6%) had tumors with PNI. There were no significant differences in age, race/ethnicity, smoking history, histologic subtype, or grade between the group of patients with PNI and the group without PNI. The group with PNI was more likely to have lichen sclerosus (25.0% vs. 15.4%, p = 0.024), stage III/IV disease (59.4% vs. 36.0%, p = 0.007), lymph node involvement (50.0% vs. 21.6%, p = 0.002), and lymphovascular space invasion (LVSI) (53.1% vs. 15.9%, p < 0.001). A higher proportion of patients in the PNI group underwent primary or adjuvant radiation therapy (68.8% vs. 45.0%, p = 0.016). The median follow-up was 67.1 months (range < 1.0 to 284.3). Patients with PNI had significantly shorter overall survival (OS), median 25.5 vs. 94.3 months (p < 0.001), and progression-free survival (PFS), median 17.5 vs. 29.0 months (p = 0.004). After adjusting for stage, patients with PNI had a greater risk for death and progression (OS: hazard ratio, 2.71; p < 0.001; PFS: hazard ratio, 1.64; p-value = 0.020).
CONCLUSION: PNI should be considered an independent poor prognostic factor for patients with vulvar carcinoma, and should be included as part of the pathologic analysis.

Lillsunde Larsson G, Kaliff M, Sorbe B, et al.
HPV16 viral characteristics in primary, recurrent and metastatic vulvar carcinoma.
Papillomavirus Res. 2018; 6:63-69 [PubMed] Free Access to Full Article Related Publications
Vulvar carcinoma is the fourth most common gynecological malignancy. Two separate carcinogenic pathways are suggested, where one is associated with the human papillomavirus (HPV) and HPV16 the most common genotype. The aim of this study was to evaluate HPV-markers in a set of primary tumors, metastases and recurrent lesions of vulvar squamous cell carcinomas (VSCC). Ten HPV16-positive VSCC with metastatic regional lymph nodes, distant lymphoid/hematogenous metastases or local recurrent lesions were investigated for HPV genotype, HPV16 variant, HPV16 viral load, HPV16 integration and HPV16 E2BS3 and 4 methylation. In all 10 analyzed case series, the same HPV genotype (HPV16), HPV16 variant and level of viral load were detected in all lesions within a patient case. Primary tumors with a high E2/E6 ratio were found to have fewer vulvar recurrences and/or metastases after diagnosis and treatment. Also, a significantly lower viral load was evident in regional lymph nodes compared to primary tumors. The data presented strengthens the evidence for a clonal HPV-induced pathway for vulvar carcinoma.

Yap J, Fox R, Narsia N, et al.
Under expression of the Sonic Hedgehog receptor, Patched1 (PTCH1), is associated with an increased risk of local recurrence in squamous cell carcinoma of the vulva arising on a background of Lichen Sclerosus.
PLoS One. 2018; 13(10):e0206553 [PubMed] Free Access to Full Article Related Publications
OBJECTIVE: Dysregulation of the Hedgehog (Hh) pathway has been described in a variety of cancers, including cervical cancer, a disease which shares a common aetiology with vulval squamous cell carcinoma (VSCC). Here, we investigate a large number of primary VSCC cases for evidence of Hedgehog pathway activation and examine the implications of pathway activity on clinical outcomes in a cohort of patients with primary VSCC.
METHODS: Archival histology blocks containing VSCC and histologically normal adjacent epithelium were retrieved from a cohort of 91 patients who underwent treatment for primary VSCC. Immunohistochemistry staining was undertaken to assess for the expression of key Hh pathway components (SHH, PTCH1, GLI1). A competing risks statistical model was used to evaluate the implications of the levels of key Hh pathway components on clinical outcomes.
RESULTS: We show that 92% of primary VSCC cases over-expressed one or more components of the Hh signalling pathway when compared to the adjacent normal epithelium. While expression of SHH and GLI1 did not correlate with any clinicopathological criteria, over- or under-expression of PTCH1 was associated with a reduced or increased risk of developing a local disease recurrence, respectively. In VSCC arising on a background of Lichen Sclerosus, the risk of local recurrence was potentiated in cases where PTCH1 was under-expressed.
CONCLUSIONS: Our findings reveal, for the first time, that the Hh pathway is activated in VSCC and that PTCH1 expression can be used as a biomarker to stratify patients and inform clinicians of the risk of their local recurrence, particularly in cases of VSCC associated with LS.

Abdulrahman Z, Kortekaas KE, De Vos Van Steenwijk PJ, et al.
The immune microenvironment in vulvar (pre)cancer: review of literature and implications for immunotherapy.
Expert Opin Biol Ther. 2018; 18(12):1223-1233 [PubMed] Related Publications
INTRODUCTION: Vulvar squamous cell carcinoma (VSCC) develops via two different pathways: TP53 mutations in a background of lichen sclerosus or a persistent infection with high-risk human papilloma virus (HPV). The latter group of tumor responds better to treatment than the non-virally induced VSCC. This may be explained by a difference in the tumor immune microenvironment (TME).
AREAS COVERED: This review summarizes literature on TME of VSCC and its precursors, and extrapolates this to foster the development of new therapeutic strategies.
EXPERT OPINION: Both types of VSCC and their precursors are infiltrated with variable numbers of M2 macrophages, regulatory T cells and CD8+ T cells, indicating that they express targetable tumor antigens. Type 1 T cell immunity in precursor lesions is associated with fewer recurrences and better clinical responses to immunotherapy. Escape of these lesions and progression toward VSCC is associated with the downregulation of HLA Class I, increased expression of co-inhibitory molecules, infiltration with immunosuppressive cells and the local production of immunosuppressive enzymes and cytokines. More in-depth studies of the VSCC TME are required to fully comprehend the impact of the immune system on VSCC, and subsequently to identify patients who will benefit from immunotherapeutic strategies.

Reda A, Gomaa I
Vulvar Lipoma: A Case Report.
Rev Bras Ginecol Obstet. 2018; 40(10):647-649 [PubMed] Related Publications
The present study is a case report of vulvar lipoma. The vulva is a rare site for the development of lipomas, and the aim of the study is to determine if the current imaging modalities can diagnose lipomas correctly. A 43-year-old patient presented with a painless, slowly progressive, oval, mobile and non-tender right vulvar mass compressing the vagina and totally covering the introitus. Both the ultrasonography and magnetic resonance imaging (MRI) exams suggested the diagnosis of lipoma. Surgical excision was performed, and the histopathological examination of the mass confirmed a lipoma.

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