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Background: Women with vulvar neoplasia continue to experience uncertainty up to six months post-surgery. Uncertainty in illness is considered a significant psychosocial stressor, that negatively influences symptom distress, self-management strategies and quality of life. According to the Reconceptualized Uncertainty in Illness Theory, the appraisal of uncertainty changes positively over time in chronic illness. We aimed at exploring whether and how the experience of uncertainty develops in women with vulvar neoplasia. Methods: We selected a purposive sample of seven women diagnosed with vulvar neoplasia in four Swiss and one Austrian women's clinic. By means of a qualitative longitudinal study, we conducted 30 individual interviews at five points of time during one year after diagnosis. We applied Saldaña's analytical questions for longitudinal qualitative research. Results: First, participants experienced uncertainty as an existential threat, then an inherent part of their illness, and finally a certainty. Women initially associated the existential threat with a high risk for suffering from severe health deteriorations. Participants that could reduce their individually assessed risk by adopting health promoting behaviors, accepted the remaining uncertainty. From now on they reframed uncertainty into a certainty. This new mindset was based on a belief of promoting recovery and reducing the risk of recurrence. Conclusions: The long-lasting and oscillating nature of uncertainty should receive attention in supportive oncology care. Uncertainty concerning existential issues is of special importance since it can inhibit a positive development of uncertainty experience. [ABSTRACT FROM AUTHOR]

In France, about 2000 new cases of anal cancer are diagnosed annually. Squamous cell carcinoma is the most common histological type, mostly occurring secondary to persistent HPV16 infection. Invasive cancer is preceded by precancerous lesions. In addition to patients with a personal history of precancerous lesions and anal cancer, three groups are at very high risk of anal cancer: (i) men who have sex with men and are living with HIV, (ii) women with a history of high-grade squamous intraepithelial lesions (HSILs) or vulvar HPV cancer, and (iii) women who received a solid organ transplant more than 10 years ago. The purpose of screening is to detect HSILs so that they can be treated, thereby reducing the risk of progression to cancer. All patients with symptoms should undergo a proctological examination including standard anoscopy. For asymptomatic patients at risk, an initial HPV16 test makes it possible to target patients at risk of HSILs likely to progress to cancer. Anal cytology is a sensitive test for HSIL detection. Its sensitivity is greater than 80% and exceeds that of proctological examination with standard anoscopy. It is indicated in the event of a positive HPV16 test. In the presence of cytological abnormalities and/or lesions and a suspicion of dysplasia on clinical examination, high-resolution anoscopy is indicated. Performance is superior to that of proctological examination with standard anoscopy. However, this technique is not widely available, which limits its use. If high-resolution anoscopy is not possible, screening by a standard proctological examination is an alternative. There is a need to develop high-resolution anoscopy and triage tests and to evaluate screening strategies. [ABSTRACT FROM AUTHOR]

Introduction: Approximately 25%–43% of all vulvar carcinomas are associated with human papillomavirus (HPV). In many countries, vulvar carcinoma incidence rates are increasing, possibly due to greater HPV exposure. However, studies exploring changes in HPV prevalence and genotype distribution in vulvar carcinoma over time are scarce. Our aim was to evaluate time trends in HPV prevalence and genotype distribution in vulvar squamous cell carcinoma in an unselected, nationwide sample of Norwegian women. Further, we explored clinical and histopathological aspects in relation to HPV status and investigated whether HPV status was associated with survival. Material and methods: All vulvar squamous cell carcinoma cases from 1970–1975 and 2000–2005 were extracted from the Cancer Registry of Norway and corresponding tissue blocks were retrieved. After detailed histology review, HPV testing was conducted using real‐time TaqMan PCR. Overall survival rates were calculated using the Kaplan–Meier method. Multivariable Cox regression analysis was performed to estimate hazard ratios adjusted for age at diagnosis, stage and diagnostic period. Results: Histological review was performed on 352 vulvar squamous cell carcinoma cases. We were able to obtain valid HPV analysis results for 282 cases, Overall, 29.8% (95% CI 24.5%–35.5%) of cases were high‐risk HPV (hrHPV)‐positive. When comparing the two periods, we found that the percentage of hrHPV‐positive tumors increased significantly from 23% (95% CI 16.0%–31.4%) in 1970–1975 to 35.3% (95% CI 27.8%–43.3%) in 2000–2005 (P = 0.025). The predominant genotypes were HPV 16 (73%), HPV 33 (21%), and HPV 18 (6%), with similar distributions in both periods. In the more recent cohort, several additional genotypes were detected: HPV 6, 11, 39, 45, 52, 58 and 66 were found in smaller percentages, ranging from 1.8% to 3.6%. In univariate analysis, patients with HPV‐positive tumors showed improved overall survival compared with patients with HPV‐negative tumors (hazard ratio [HR] 0.65, 95% CI 0.48–0.86). Conclusions: The prevalence of HPV in vulvar squamous cell carcinomas in Norway was significantly higher in 2000–2005 than in 1970–1975. The three predominant genotypes were HPV 16, 33 and 18 in both time periods. However, several other HPV genotypes have emerged over the last decades. HPV‐positivity was associated with better overall survival. [ABSTRACT FROM AUTHOR]

Objective: The current study was performed to evaluate the incidence of positive lymph nodes (LNs) in relation to known pathological risk factors, specifically among patients with apparent low‐grade, small tumors. Methods: We used the Surveillance, Epidemiology, and End Results (SEER) database to retrospectively identify patients with vulvar squamous cell carcinoma (SCC) diagnosed between January 1, 2000, and December 31, 2019, with known tumor size and regional LN examined. A comparison between patients who had positive and negative LNs was conducted to identify risk factors for LN metastases in relation to survival. Subgroup analysis was conducted in patients with diagnosed grade 1 vulvar SCC and tumor size up to 2 cm according to the status of LNs. Results: Multivariate analysis found that both grade of disease and tumor size were significant factors in predicting LN status. Among patients with low‐grade small tumors up to 2 cm, the odds ratio for positive LNs was 2.5 for those with tumor size larger than 1 cm. In a multivariate survival analysis, older age, larger tumor size, and positive LNs were independently associated with decreased survival. Conclusions: The current study confirms that among small tumors, those larger than 1 cm have a significantly increased risk for positive nodes compared with those smaller than 1 cm, and, among this specific group, patients with positive nodes have decreased survival. Future studies are needed to answer the question of whether, in the era of the sentinel node procedure, it is safe to omit LN evaluation altogether. Synopsis: Patients with small vulvar cancer tumors up to 1 cm present with decreased rates of lymph node metastases, and patients with positive nodes have decreased survival. [ABSTRACT FROM AUTHOR]

Introduction: Liposarcoma of the vulva is extremely rare, it represents a diagnostic challenge for the gynecologist as well as the pathologist, there are few cases reported in the literature. We present a case of pleomorphic liposarcoma of the vulva, the first reported at the National Oncological Institute in Panama. Case report: This is a 52-year-old woman with a one-year history of a tumor on the right labia majora of the vulva, with accelerated growth in recent months associated with ulceration. The biopsy reported high-grade malignant mesenchymal neoplasia. She was taken After surgery, hemivulvectomy and flap reconstruction, the resection margins were negative. Postoperative evolution without adverse events. Conclusions: Pleomorphic liposarcoma of the vulva is an extremely rare entity; biopsy of the vulvar tumor is crucial for planning surgery, the treatment of choice in most cases. Adjuvant treatment is individualized and follow-up could be close. [ABSTRACT FROM AUTHOR]

Vulvar intraepithelial neoplasia (VIN), the precursor lesion of vulvar squamous cell carcinoma (VSCC), may present as pruritic or asymptomatic lichenified plaques surrounded by single or multiple discrete or confluent macules or papules. VIN is divided into high-grade squamous intraepithelial lesion (HSIL), which is human papillomavirus (HPV)-driven, and differentiated VIN (DVIN), which develops independently of HPV. Histopathological examination and HPV genotyping polymerase chain reaction (PCR) tests should be performed to distinguish between HSIL and DVIN. Lichenified plaques surrounded by multiple papules are found not only in VIN but also in vulvar lichen simplex chronicus (LSC). This chronic inflammatory skin disease mostly appears in labia majora and is triggered by sweating, rubbing, and mental stress. IHC staining of p16 and p53 are recommended as the most commonly used biomarkers for VIN in diagnostically challenging cases. IHC staining is also beneficial to confirm the accuracy of the HPV detection technique, as p16-negative staining may also represent a false-positive result. We report a case of the importance of p16 and p53 IHC staining in diagnosing vulvar LSC mimicking VIN with false-positive HPV-66. The patient was previously diagnosed with VIN based on clinical examination. HPV-66 was detected by PCR from a vulvar biopsy sample. Histopathological examination revealed stromal lymphocytic infiltration with non-specific chronic dermatitis; neither atypia nor koilocyte was observed. Both p16 and p53 IHC staining were negative. The patient was diagnosed and treated as vulvar LSC with 10 mg cetirizine tablet, emollient, and 0.1% mometasone furoate cream. Clinical improvement was observed as the lesions became asymptomatic hyperpigmented macules in the 4 weeks of follow-up, without recurrence after 3 years of follow-up. Both p16 and p53 IHC staining might help distinguish HSIL and DVIN mutually and from other vulvar mimics in diagnostically challenging cases. [ABSTRACT FROM AUTHOR]

The greatest challenge in the management of vulvar squamous cell carcinoma (VSCC) is treatment of recurrent disease where options for surgery and radiation have been exhausted, or treatment of disease where distant metastasis is present. Identification of mutations differentially expressed between tumor from patients who died of aggressive disease and tumor from patients with an indolent course could reveal novel prognostic indicators and guide development of therapeutic drugs. From 202 consecutive patients with VSCC, patients who recurred and died of disease (group A) were identified and matched by age, tumor size, depth of invasion and nodal status with those whose disease did not recur (group B). Tumors from 21 patients were subjected to whole exome sequencing of DNA and RNA, immunohistochemistry (IHC) antibodies of PD-L1 and P16, and in-situ hybridization (ISH) for high-risk HPV. Analysis of DNA and RNA revealed six genes that were strongly differentially expressed between group A and B: TGM3 , ACVR2A , ROS1 , NFEL2 , CCND1 and BCL6. Clinically relevant DNA mutations were significantly greater in group A versus B: 7 vs 2.3 mutations per patient. The most common genomic alterations were mutations in TP53 and the promoter region of TERT. Other common genomic events include alterations of FAT1 , CDKN2A , PIK3CA , CCND1 , and LRP1B. All samples were MSI stable and tumor mutational burden (TMB) was similar in groups A and B. Most VSCC specimens (81%) were positive for PD-L1. ACVR2A and TGM3 are significantly under-expressed in tumors with poor outcome, suggesting they may play a role in tumor suppression. Clinical outcome of VSCC appears independent of MSI, TMB, or PD-L1 status. • Vulvar squamous cell carcinoma (VSCC), a heterogeneous disease, has few prognostic indicators. • Whole exome sequencing identifies TGM3 and ACVR2 genes as significantly under-expressed in VSCC tumors with poor outcome. • The frequency of DNA mutations was observed to associate with a higher mortality. • Clinical outcome of VSCC may be independent of MSI, TMB, or PD-L1 status. [ABSTRACT FROM AUTHOR]

In order to retrospectively analyse the multi‐site involvement pattern of erosive lichen planus patients, we retrospectively reported the clinical and medical data of three patients with erosive lichen planus which involving their vulva, vagina, gingiva, and ear canal. We confirmed the existence of otic lichen planus, and found that it is more common in patients with vulvovaginal‐gingival syndrome of erosive lichen planus. Therefore, we propose 'vulvovaginal‐gingival‐otic syndrome' to further describe this rare compound pattern of lichen planus. [ABSTRACT FROM AUTHOR]

Introduction: Vaginal cancer is a rare gynecologic malignancy. While in a localized disease, concurrent chemoradiation grants local control and better overall survival, in a metastatic setting, the management options are very limited. Furthermore, recurrent cervical, vulvar, and vaginal carcinomas notoriously develop resistance to treatment, and consequently, their prognosis is still poor. Case Presentation: We herein present the case of a woman with a nodal relapse of vaginal carcinoma, effectively treated with third-line immunotherapy. We will also provide a review of the literature on the new therapeutic strategies for advanced vaginal carcinoma, with a focus on pembrolizumab immunotherapy. Conclusion: Pembrolizumab might represent a promising option for the management of vaginal and vulvar cancer, but data to support its use in this setting are still lacking. This case highlights the need for further investigation and trial designs for this rare disease. [ABSTRACT FROM AUTHOR]

Background/Objectives: Pediatric vulvar disease has not been widely explored in the medical literature. Few studies focus on vulvar disease in skin of color. The vulvar disease can be distressing for young patients given the sensitive location, and providers may lack experience in diagnosing and managing vulvar dermatoses. We sought to characterize the conditions seen, diagnostic challenges encountered, and the racial and ethnic factors associated with vulvar diseases in our multidisciplinary pediatric dermatology‐gynecology vulvar clinic at Children's National. Methods: Medical records of 220 patients who presented to our multidisciplinary pediatric dermatology‐gynecology clinic were reviewed retrospectively. Results: Lichen sclerosus (LS) (36%, n = 80), inflammatory vulvitis (11%, n = 23), and vitiligo (9%, n = 19) were the three most frequent conditions observed. These conditions were often misdiagnosed as one another. There was a mean delay in diagnosis after symptom onset in LS patients of 16.43 months. Conclusions: LS, inflammatory vulvitis, and vitiligo are common vulvar diseases among pediatric patients. Accurate diagnosis is important because LS must be treated aggressively to prevent sequelae. Further studies are warranted to help differentiate LS and vitiligo with consideration of skin tone. [ABSTRACT FROM AUTHOR]

Opinion Statement: Sentinel lymph node mapping (SLNM) and dissection (SLND) should be used as an alternative to full inguinofemoral lymph node dissection (IFLND) in select patients with early-stage vulvar cancer. IFLND is associated with high postoperative complications such as wound breakdown, lymphedema, lymphocyst formation, and infection. SLND in select patients offers a safe, effective, and less morbid alternative. Candidates for SLND include patients with a unifocal vulvar tumor less than four centimeters, clinically negative lymph nodes, and no prior inguinofemoral surgeries. SLND should ideally be performed by a high-volume SLN surgeon. Most commonly, SLND is performed using both radiocolloid lymphoscintigraphy (e.g., Technetium-99) and a visual tracer such as blue dye; however, near infrared imaging with indocyanine green injection is becoming more widely adopted. Further prospective studies are needed to examine the safety and efficacy of various techniques for SLND. SLND has been demonstrated to be cost-effective, especially when including perioperative complications. Further studies are needed to demonstrate quality of life differences between IFLND and SLND. [ABSTRACT FROM AUTHOR]

Oncovirus infections account for an estimated 12%−20% of human cancers worldwide. High‐risk human papillomavirus (HPV) infection is the etiological agent of some malignancies such as cervical, oropharyngeal, anal, penile, vaginal, and vulvar cancers. However, HPV infection is not the only cause of these cancers or may not be sufficient to initiate cancer development. Actually, certain other risk factors including additional oncoviruses coinfections have been reported to increase the risk of patients exposed to HPV for developing different HPV‐related cancers. In the current review, we summarize recent findings about coinfections with different oncoviruses in HPV+ patients from both clinical and mechanistic studies. We believe such efforts may lead to an interesting direction for improving our understanding and developing new treatments for virus‐induced cancers. [ABSTRACT FROM AUTHOR]

Vulvar cancer remains a significant health concern for women worldwide, it provides a comprehensive overview of biomarkers in vulvar cancer, focusing on the significance of EGFR, GLUT1, MUC1, and MRP1. It explores their prognostic value, pathophysiology and delves into the intricate landscape of P16, PD-L1, and P53 biomarkers. The article aims to elucidate the roles of these biomarkers in predicting outcomes, offering insights into their potential as prognostic indicators for vulvar cancer. This analysis contributes to the evolving understanding of molecular markers in the context of vulvar cancer prognosis and informs future research directions in the field. [ABSTRACT FROM AUTHOR]

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Background: Recent calls to action highlight the need to address gaps in our understanding of survivorship for those living with advanced gynecological cancer to support optimal care. To ensure future research fills these knowledge gaps, we need to understand the breadth of existing survivorship research in this patient group, including the outcomes assessed, the populations included and the duration and retention in follow‐up. Methods: We conducted a systematic scoping review searching PubMed, PsychINFO, and CINAHL during the month of November 2022 to identify prospective cohort studies measuring survivorship outcomes among participants with advanced (stage III–IV) gynecological cancer, or in cohorts in which ≥50% of participants had advanced cancer, or which provide results separately for patients with advanced cancer. Articles were screened, and data extracted using a standard form. Results: We assessed 33 articles from 21 unique studies, which overall included 6023 participants with gynecological cancer. Of these, 45% had cervical cancer, 44% ovarian, 10% endometrial/uterine, and 1% vaginal/vulvar cancer. The most frequently measured survivorship outcome was quality of life. Of the 33 articles, most reported on participant age (n = 31), but relatively few reported on comorbidities (n = 10), physical status (n = 6), ethnic background (n = 4), the country of birth (n = 2), or the area of participant residence (n = 2). None included details on indigenous status. Recruitment proportions ranged from 48% to 100%. Retention proportions ranged from 15% to 97%. Conclusion: Our findings highlight gaps in survivorship research for advanced gynecological cancers and emphasize the need for future studies to include and describe the experiences of diverse and underrepresented groups. [ABSTRACT FROM AUTHOR]

Simple Summary: In view of the high recurrence rates of vulvar cancer, often even after successful surgery, this article emphasises the crucial role of radiotherapy. Surgery remains the primary treatment, but radiotherapy, particularly brachytherapy (BT) either alone or in combination with external beam radiotherapy (EBRT), plays an important role in the adjuvant and primary treatment of vulvar cancer. The article highlights the advantages of BT, including improved dose precision, minimised impact on organs at risk and support for hypofractionated accelerated treatment. This narrative review provides recent data on the importance of BT in the treatment of primary and recurrent vulvar cancer, covering radiobiological, clinical and therapeutic aspects. Vulvar cancer is a relatively rare neoplasm. The essential treatment is surgery for the primary tumour. However, postoperative recurrence rates are high, even in early-stage disease when tumour-free surgical margins are achieved or in the absence of associated risk factors (lymph node metastases, deep stromal invasion or invasion of the lymphatic vascular space). Radiotherapy plays an important role in the treatment of vulvar cancer. Adjuvant treatment after surgery as well as primary treatment of locally advanced vulvar cancer (LAVC) is composed of two key radiotherapy treatment scenarios, external beam radiation therapy (EBRT) either combined or not combined with brachytherapy (BT). In a recurrence setting, where surgery is not an option, BT alone or in combination with EBRT can be used. Compared to EBRT, BT has the radiobiological potential to improve dose to the target volume, minimise the dose to organs at risk, and facilitate hypofractionated-accelerated treatment. This narrative review presents recent data on the role of BT in the treatment of primary and/or recurrent vulvar cancer, including radiobiological, clinical, and therapeutic aspects. [ABSTRACT FROM AUTHOR]

Objective: Central nervous system (CNS) metastasis originating from gynecological cancer is a very rare and late manifestation of the disease. Therefore, there is still limited data on prognostic factors for survival. The objective of the present study is to identify prognostic factors for survival in patients with CNS metastasis originating from gynecological cancer. Study Design: The present retrospective study analyzed the patients with gynecological cancers who were treated due to CNS metastases between January 1999 and December 2019 at Istanbul University Hospital. Results: Forty-seven patients with CNS metastasis of gynecological origin were included in the study. The median age at the time of CNS metastasis was 59 (range 34–93). The median time from initial cancer diagnosis to CNS metastasis was 24.9 (range: 0–108.2) months. Most patients had epithelial ovarian cancer (EOC) (76.6%), followed by endometrial cancer (EC) (14.8%), cervical cancer (CC) (4.3%), and vulvar cancer (VC) (4.3%). By multivariate analysis, the presence of extracranial metastasis (HR: 5.10; 95% CI: 1.71-15.18), Eastern Cooperative Oncology Group (ECOG) performance status ≥3 (HR: 2.92; 95% CI: 1.36-6.26), palliative care only for the treatment of CNS metastasis (HR: 1.47; 95% CI: 0.58-4.11), and treatment-free interval (TFI) <6 months (HR: 2.74; 95% CI: 1.23-6.08) were independent factors that associated with worse survival. Conclusion: Patients with CNS metastasis who have favorable prognostic factors are considered to be appropriate candidates for aggressive and long-term treatment strategies. Extracranial metastasis, ECOG performance status, treatment history of CNS metastasis, and TFI were determined as independent prognostic factors that improved survival. TFI might be taken into account as a prognostic factor for patients with CNS metastasis in gynecological cancer. [ABSTRACT FROM AUTHOR]

This article discusses a rare case of hidradenoma papilliferum (HP), a benign tumor, in the perineum of a virgin woman. HP is typically found in the anogenital region of female patients between the ages of 20 and 90, but it can also occur in other areas of the body. The diagnosis of HP can be challenging due to its similarity to other benign tumors, and the prevalence and incidence of the condition are unknown. Treatment involves complete excision of the tumor, and histopathological examination is necessary for an accurate diagnosis. While HP is generally asymptomatic, this particular case presented with itching and bleeding. The article also discusses the clinical features, differential diagnosis, and treatment options for HP. [Extracted from the article]

Despite being a disease of mainly older women, little is known about the clinical management of older women with vulvar squamous cell carcinoma (VSCC). We evaluated their daily clinical management compared with younger women, and established the prevalence of comorbidities and its impact on overall survival (OS). All Dutch women diagnosed with VSCC from 2015 to 2020 (n = 2249) were selected from the Netherlands Cancer Registry. Women aged ≥80 years (n = 632, 28%) were defined as "older" patients, women <80 years were considered as "younger". Chi-square tests were performed to evaluate differences in treatment by age group and comorbidities. Differences in OS were evaluated using Kaplan-Meier Curves and log-rank test. The vast majority of both older (91%) and younger (99%) patients with FIGO IA VSCC received surgical treatment of the vulva. Older FIGO IB-IV VSCC patients were less likely to undergo groin surgery than younger patients (50% vs. 84%, p < 0.01). Performance of surgical treatment of the vulva and groin(s) was not associated with the number of comorbidities in older patients (p = 0.67 and p = 0.69). Older patients with ≥2 comorbidities did have poorer OS compared to women with one or no comorbidities (p < 0.01). The vast majority of older patients underwent vulvar/local surgery. Older patients less often received groin surgery compared to younger patients. The majority of older patients had at least one comorbidity, but this did not impact treatment choice. The poorer survival in older VSCC patients may therefore be due to death of competing risks instead of VSCC itself. • VSCC is primarily a disease of older women, with 28% being aged ≥80 years • Older patients (≥80 years) less often received groin surgery than younger patients • 63% of the older patients had at least one comorbidity, 25% had ≥2 comorbidities • Treatment of older VSCC patients was not related to the number of comorbidities • Only older patients with ≥2 comorbidities did have a poorer overall survival [ABSTRACT FROM AUTHOR]

The article discusses the neglect of vulvovaginal diseases in medical training and practice, highlighting the lack of exposure and training in managing these conditions. It explores the potential of artificial intelligence (AI) in improving the diagnosis and management of vulvovaginal diseases, particularly focusing on vulvar lichen sclerosus (VLS). The authors emphasize the importance of early diagnosis, proper treatment, and follow-up to reduce the risk of vulvar cancer, suggesting that AI and molecular testing could play a significant role in the future of healthcare. The article underscores the need for a balance between technological advancements like AI and the human factor in providing empathetic and effective care for patients with vulvovaginal diseases. [Extracted from the article]

Vulvar intraepithelial neoplasia, also known as VIN, is a non-invasive squamous lesion and precursor of squamous cell carcinoma (SCC) of the vulva. There is no screening test for vulvar intraepithelial neoplasia. Diagnosis of VIN is made clinically and confirmed with a biopsy. We describe a 66-year-old woman with a condyloma-like tumour located in the skin on the vestibule of the vagina. A biopsy sample was taken from the nodule. The definitive diagnosis is supported by the histological examination (VIN III) and immunohistochemical examination of p16(+), p53(+), and a few cell nuclei. The case provides information on the importance of multidisciplinary cooperation. Lifelong surveillance is essential since the resection of individual lesions does not guarantee the prevention of invasive cancer.

IntroductionVulvar cancer carries a favourable prognosis in early stages. However, therapeutic options for advanced or recurrent cases are limited despite a variety of therapeutic modalities, such as extensive surgical resection, chemotherapy, and radiotherapy. The most important emerging treatment modalities are immune checkpoint inhibitors. This systematic review and meta-analysis aims to assess the efficacy and safety of pembrolizumab, an immune checkpoint inhibitor, in women with advanced vulvar cancer.Materials and methodsFollowing a comprehensive search, review, and appraisal, two relevant single-arm studies were included. Meta-analysis was conducted using R4.3.0 software and RStudio 2023.03.0, presenting the overall effect size with a 95% confidence interval. Heterogeneity was assessed using I2 and the Cochrane Q χ2 statistics.ResultsOut of 154 studies screened for eligibility, two single-arm studies involving 119 patients receiving pembrolizumab for advanced vulvar cancer were included. The pooled objective response rate (ORR) was overall 10% (95% CI: 0.00-0.84) and 9% (95% CI: 0.00-0.89) in the PD-L1 positive subgroup. In the intention-to-treat (ITT) population, 31% (95% CI: 0.04-0.85) exhibited any clinical benefit (complete response, partial response, or stable disease). In the ITT population at six months, progression-free survival (PFS) was 19% (95% CI: 0.01-0.82), and overall survival (OS) was 48% (95% CI: 0.08-0.90). At 12 months, PFS decreased to 9% (95% CI: 0.00-0.85), and OS was 33% (95% CI: 0.04-0.85). No statistically significant heterogeneity was observed in PFS and OS analyses.Discussion and conclusionThis study suggests that one-third of women with advanced or recurrent vulvar cancer may, without the influence of PD-L1 status, benefit from pembrolizumab treatment despite a decline in both PFS and OS at 12 months. These findings provide support for considering pembrolizumab in the treatment paradigm for this specific subset of cancer patients.Systematic review registrationhttps://www.crd.york.ac.uk/prospero/, identifier CRD42023391888

This article discusses a case of carcinoma in situ arising in a patient with Hailey-Hailey disease (HHD), a rare inherited blistering disorder. The patient presented with recurrent itchy lesions on the scrotum and groin folds, along with a growth on the scrotum associated with pus discharge and bleeding. The diagnosis of carcinoma in situ was confirmed through histopathological examination, and a wide local excision was performed. The article highlights the occurrence of this condition in HHD and discusses the possible factors contributing to the development of cutaneous malignancies in HHD. [Extracted from the article]

This document is a letter published in the Archives of Gynecology & Obstetrics. It discusses a bibliometric analysis of the most cited papers in gynecologic oncology, aiming to provide insight into the future direction and development of the field. The study identified the top-cited articles per year in the Journal of Gynecologic Oncology using the Web of Science and iCite databases. The most frequently cited article was a basic science study on MicroRNA signatures of tumor-derived exosomes as diagnostic biomarkers of ovarian cancer. The analysis also revealed that observational studies were the most common study methodology, and ovarian cancer was the most common study topic. The majority of the articles were authored by researchers from the United States. The letter emphasizes the importance of basic science research in gynecologic oncology and highlights the need for international and multi-center collaborations in oncology studies. [Extracted from the article]

This article discusses a rare case of concurrent vulvar Crohn's disease (CD) and pyoderma gangrenosum (PG). CD is a chronic inflammatory condition that affects the gastrointestinal tract, and vulvar involvement is a rare extraintestinal manifestation. PG is an autoinflammatory dermatitis that is infrequently seen in patients with CD. The article presents the case of a 33-year-old female with vulvar CD and PG, describing her symptoms, physical exam findings, and treatment. The article emphasizes the need for a multidisciplinary approach to care and mentions various treatment options for vulvar CD and PG. [Extracted from the article]

A 54-year-old woman with a history of cervical cancer presented with a 6-year history of vaginal papules and vesicles that oozed serous fluid. She had lesions both in the field of radiation and away from it, as well as lower extremity swelling and recurrent episodes of lower abdominal cellulitis. A biopsy confirmed the diagnosis of acquired lymphangiectasia of the vulva (ALV) secondary to radiotherapy. Treatment options for ALV include surgical excision, labial reduction, CO2 laser therapy, or superficial radiotherapy. [Extracted from the article]

Obliterative surgical procedures have been classically used as a method to reduce pelvic organ prolapse in elderly women who do not wish to preserve the vagina for sexual intercourse. The aim of this video is to demonstrate a surgical technique of repairing complete labia majora fusion in a woman who had previously undergone one of these procedures. We present the case of an 80 year-old woman with a history of progressive difficulty on voiding onset and sensation of incomplete bladder emptying. She reported an obliterative procedure to correct her pelvic organ prolapse (POP) 10 years earlier. On physical examination, complete fusion of labia majora was observed, causing abnormal urinary drainage. Perineal reconstructive surgery was performed without complications and the application of topical Promestriene was prescribed. Optimal genital re-epithelization was observed during follow-up. As observed, patients undergoing obliterative surgery who present with genital atrophy are at increased risk of developing vulvar adherences. Applying vaginal moisturizers or local topical estrogens can prevent this condition. In severe vulvar fusions, early surgical treatment is recommended to prevent potentially serious complications. [ABSTRACT FROM AUTHOR]

This article discusses the case of a 7-year-old girl with vulvar syringomas, which are benign tumors arising from the ducts of the eccrine glands. Vulvar syringomas are rare in children, and they typically present with pruritus (itching). The article explores possible mechanisms for the pruritus, including friction, inflammation, maceration, occlusion, and hormonal factors. Treatment options for vulvar syringomas include destructive treatments and medical treatments, although there is no definitive cure. The authors recommend histopathological examination to confirm the diagnosis and suggest non-fluorinated topical corticosteroids as a potential treatment option for pruritus. [Extracted from the article]

The article focuses on recent developments in vulvar lichen sclerosus (VLS), updates such as the establishment of core outcome domains for clinical trials, increased awareness of VLS's impact on quality of life, and advancements in disease associations and management strategies. It highlights the importance of standardized outcome measurements in future clinical trials to assess efficacy and safety of new treatments for VLS.

Secondary osteomyelitis pubis is rare, particularly when it arises due to genitourinary postoperative infections, such as those occurring after vulvar cancer surgeries. Diagnosis and treatment of secondary osteomyelitis pubis are challenging. Here, we report on two cases of osteomyelitis pubis caused by Pseudomonas aeruginosa secondary to surgical site infections after of vulvar cancer surgeries. Both patients were in their 80 s and underwent vulvectomy and vulvar reconstructive surgery using skin flaps. The patients were discharged from the hospital after postoperative antimicrobial treatment for surgical site infections and continued self-cleaning of the wound dehiscence. Both patients presented, respectively, with gait disturbance due to pain in the pubic bone postoperatively at 24 and 7 weeks. Computed tomography (CT) and magnetic resonance imaging (MRI) were performed to confirm the diagnosis of osteomyelitis pubis. The patients underwent pubic bone debridement, and tissue culture revealed the presence of Pseudomonas aeruginosa that required several months of antimicrobial therapy. Pubic pain and gait disturbance improved with treatment, and no osteomyelitis pubis relapse has been observed in both cases 12 and 9 months since treatment initiation. CT and MRI were useful in diagnosing osteomyelitis pubis. Early debridement helped identify the causative organism and appropriate antibiotics selection.

ObjectiveTo assess quality of life (QOL) in patients who developed lower-extremity lymphedema (LLE) after radical gynecologic cancer surgery on prospective clinical trial GOG 244.MethodsThe prospective, national, cooperative group trial GOG-0244 determined the incidence of LLE and risk factors for LLE development, as well as associated impacts on QOL, in newly diagnosed patients undergoing surgery for endometrial, cervical, or vulvar cancer from 6/4/2012-11/17/2014. Patient-reported outcome (PRO) measures of QOL (by the Functional Assessment of Cancer Therapy [FACT]), body image, sexual and vaginal function, limb function, and cancer distress were recorded at baseline (within 14 days before surgery), and at 6, 12, 18, and 24 months after surgery. Assessments of LLE symptoms and disability were completed at the time of lower limb volume measurement. A linear mixed model was applied to examine the association of PROs/QOL with a Gynecologic Cancer Lymphedema Questionnaire (GCLQ) total score incremental change ≥4 (indicative of increased LLE symptoms) from baseline, a formal diagnosis of LLE (per the GCLQ), and limb volume change (LVC) ≥10%.ResultsIn 768 evaluable patients, those with a GCLQ score change ≥4 from baseline had significantly worse QOL (p

Vulvar cancer is an uncommon malignancy. Vulvar cancer alarmed the public health problem in terms of the cost of diagnostic and medical treatments and psychical health of females. Our study aims to provide a thorough analysis of the global disease burden, related risk factors and temporal incidence trends of vulvar cancer in population subgroups. Data from Global Cancer Observatory and the Cancer Incidence in Five Continents Plus were used for the vulvar cancer incidence. Age‐standardized rates (ASR) were used to depict the incidence of vulvar cancer. The 10‐year trend of incidence was assessed using joinpoint regression with average annual percentage change and 95% confidence intervals in various age groups, while its correlations with risk factors were investigated using linear regression. Higher ASR were found in Western Europe (2.4), Northern America (1.9), Northern Europe (1.9), Australia and New Zealand (1.8) and Eastern Africa (1.4). The associated risk factors of higher vulvar cancer incidence were gross domestic product per capita, Human Development Index, higher prevalence of smoking, alcohol drinking, unsafe sex and human immunodeficiency virus infection. The overall trend of vulvar cancer incidence was increasing. An increasing trend was found in older females while a mixed trend was observed in younger females. The disease burden of vulvar cancer follows a bimodal pattern according to its two histologic pathways, affecting women in both developed and developing regions. Smoking cessation, sex education and human papillomavirus vaccination programs should be promoted among the general population. Subsequent studies can be done to explore the reasons behind the increasing trend of vulvar cancer. [ABSTRACT FROM AUTHOR]

Objective: To elucidate clinical characteristics and build a prognostic nomogram for patients with vulvar cancer. Methods: The study population was drawn from the Surveillance, Epidemiology, and End Results (SEER) database. Patients were randomly assigned to training and validation sets. Cox proportional hazards model and competing risk model were used to identify the prognostic parameters of overall survival (OS) and cancer-specific survival (CSS) to construct a nomogram. The nomogram was assessed by concordance index (C-index), area under the curve (AUC), calibration plot, and decision curve analysis (DCA). Results: A total of 20,716 patients were included in epidemiological analysis, of whom 7,025 patients were selected in survival analysis, including 4,215 and 2,810 in training and validation sets, respectively. The multivariate Cox model showed that the predictors for OS were age, marital status, histopathology, differentiation and tumor node metastasis (TNM) stages, whether to undergo surgery and chemotherapy. However, the predictors for CSS were age, race, differentiation and TNM stages, whether to undergo surgery and radiation. The C-index for OS and CSS in the training set were 0.76 and 0.80. The AUC in the training set for 1-, 3- and 5-year OS and CSS were 0.84, 0.81, 0.80 and 0.88, 0.85, 0.83, respectively, which was similar in the validation set. The calibration curves showed good agreement between prediction and actual observations. DCA revealed that the nomogram had a better discrimination than TNM stages. Conclusions: The nomogram showed accurate prognostic prediction in OS and CSS for vulvar cancer, which could provide guidance to clinical practice. [ABSTRACT FROM AUTHOR]

Objective: Extramammary Paget's disease (EMPD) of the vulva is a rare disease which predominantly presents in postmenopausal Caucasian women. As yet, no studies on Asian female patients with EMPD have been performed. This study aimed to identify the clinical features of patients with vulvar EMPD in Korea, and to evaluate the risk factors of recurrence and postoperative complications in surgically treated EMPD. Methods: We retrospectively reviewed 47 patients with vulvar EMPD who underwent wide local excision or radical vulvectomy. The clinical data and surgical and oncological outcomes following surgery were extracted from medical records and analyzed. Univariate and multivariate analyses for predicting recurrence and postoperative complications were performed. Results: 21.3% of patients had complications after surgery, and wound dehiscence was the most common. 14.9% of patients experienced recurrence, and the median interval to recurrence from initial treatment was 69 (range 33-169) months. Vulvar lesions larger than 40 mm was the independent risk factor of postoperative complications (odds ratio [OR]=7.259; 95% confidence interval [CI]=1.545-34.100; p=0.012). Surgical margin status was not associated with recurrence in surgically treated vulvar EMPD patients (OR=0.83; 95% CI=0.16-4.19; p=1.000). Conclusion: Positive surgical margin is a frequent finding in the patients with vulvar EMPD, but disease recurrence is not related with surgical margin status. Since EMPD is a slow growing tumor, a surveillance period longer than 5 years is required. [ABSTRACT FROM AUTHOR]

Vulvar squamous cell carcinoma with sarcomatoid features is an extremely rare histological variant of squamous cell carcinoma with co-existence of both epithelial and mesenchymal features. A 70-year-old woman presented with genital lesions for 4 months with associated burning and pain. Examination revealed well-defined bilaterally symmetrical hyperpigmented plaques on labia majora, fleshy erythematous growth on labia minora. Biopsy from the fleshy mass was suggestive of sarcomatoid malignancy. However, immunohistochemistry was positive for cytokeratin AE1/AE3 and negative for desmin and smooth muscle actin. Biopsy from hyperpigmented plaque was suggestive of Bowens disease. On the basis of histopathology and immunohistochemistry findings, diagnosis of vulvar sarcomatoid squamous cell carcinoma with Bowens disease was made and patient was started on external beam radiation therapy. Sarcomatoid squamous cell carcinoma of vulva is very rare cancer that has an aggressive and fatal course. Diagnosis has traditionally been difficult due to a large ratio of sarcomatous to squamous cell component. Due to its rarity, there are no distinct guidelines to direct therapy and care. [ABSTRACT FROM AUTHOR]

Purpose: More than 99% of cervical cancers and up to 40% of vulvar cancers are human papillomavirus (HPV) related. HPV 16 and 18 are the most relevant subtypes. Novel technologies allow the detection of minimal amounts of circulating cell-free HPV DNA (ccfHPV-DNA). The aim of this study was to evaluate ccfHPV-DNA assessed by droplet digital PCR (ddPCR) as a biomarker for molecular therapy monitoring in early, advanced, relapsed and metastatic HPV-driven cervical and vulvar cancer. Methods: Inclusion criteria of the study were histologically proven HPV 16/18-driven cervical and vulvar cancer with first diagnosed disease, newly diagnosed recurrence, or progression of disease. Blood samples were taken pre- and post-therapeutically. Circulating cell-free HPV DNA was quantified using ddPCR and the results were correlated with clinical data. Results: The mean copy number of ccfHPV-DNA was 838.6 (± 3089.1) in pretreatment and 2.3 (± 6.4) in post-treatment samples (p < 0.05). The copy number of ccfHPV-DNA increased with higher FIGO stages (p < 0.05), which are commonly used for clinical staging/assessment. Furthermore, we compared the distribution of copy numbers between T-stage 1 versus T-stage 2/3. We could show higher copy number level of ccfHPV-DNA in T-stage 2/3 (p < 0.05). Conclusions: Therapy monitoring with determination of ccfHPV-DNA by ddPCR with a small amount of plasma reflects response to therapy and appears feasible for patients in advanced cancer stages of cervical and vulvar cancer. This promising tool should be examined as marker of therapy monitoring in particular in novel HPV-directed therapies. [ABSTRACT FROM AUTHOR]

Human papillomavirus (HPV) encompasses a group of viruses that infect the skin and mucous membranes. In the presence of certain factors, persistent infection with high‐risk HPVs can trigger a process of neoplastic transformation. Imiquimod is a topical agent that acts as a Toll‐like receptor 7/8 agonist, stimulating the innate and adaptive immune system to exert antitumor and antiviral effects. It has been approved for the treatment of various skin conditions, however, its efficacy and safety in the management of HPV‐related‐neoplasms of the lower genital tract, such as vulvar, vaginal, and cervical neoplasia, are still under investigation. This review summarizes the current evidence on the use of imiquimod for the treatment of HPV‐induced lesions of the female lower genital tract, focusing on its indications, mechanisms of action, outcomes, and predictors of response. [ABSTRACT FROM AUTHOR]

Sentinel lymph node biopsy (SLNB) has been widely adopted in the management of early-stage gynaecological cancers such as endometrial, vulvar and cervical cancer. Comprehensive surgical staging is crucial for patients with early-stage ovarian cancer and currently, that includes bilateral pelvic and para-aortic lymph node assessment. SLNB allows the identification, excision and pathological assessment of the first draining lymph nodes, thus negating the need for a full lymphadenectomy. We systematically searched the MEDLINE, Embase and Cochrane Central Register of Controlled Trials (CENTRAL) databases (from inception to 3 November 2022) in accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA). Our search identified 153 articles from which 11 were eligible for inclusion. Patients with clinical stage I–II ovarian cancer undergoing sentinel lymph node biopsy were included. Statistical analysis was performed in RStudio using the meta package, where meta-analysis was performed for the detection. The risk of bias was assessed using the Quality Assessment of Diagnostic Accuracy Studies C (QUADAS-C) tool. Overall, 11 observational studies met the predetermined criteria and these included 194 women. The meta-analysis showed that the detection rate of sentinel lymph nodes in early-stage ovarian cancer was 94% (95% CI of 86% to 1.00%). Significant heterogeneity was noted among the studies with Q = 47.6, p < 0.0001, I2 = 79% and τ2 = 0.02. Sentinel lymph nodes in early-stage ovarian cancer have a high detection rate and can potentially have applicability in clinical practice. However, considering the small number of participants in the studies, the heterogeneity among them and the low quality of evidence, the results should be interpreted with caution. Larger trials are needed before a change in clinical practice is recommended. [ABSTRACT FROM AUTHOR]

(1) Background: Surgical-oncological treatment methods are continuously put to the test in times of evidence-based medicine—notably, a constant reevaluation remains key, especially for tumor entities with increasing incidence such as vulvar carcinoma. (2) Methods: In order to determine the postoperative clinical course of different methods of vulvar excision (vulvectomy, hemivulvectomy) as well as inguinal lymph node removal (lymphadenectomy, sentinel lymph node biopsy) with regard to postoperative wound-healingprocess, perioperative hemorrhage, and re-resection rates, we retrospectively analyzed surgical, morphological and laboratory data of 76 patients with a pathological diagnosed vulvar cancer. (3) Results: Analysis of our data from a single center revealed a comparable perioperative clinical course regardless of the chosen method of vulvar excision and inguinal lymph node removal. (4) Conclusions: Thus, our results emphasize the current multimodality in surgical therapy of vulvar carcinoma, in which consideration of known prognostic factors together with the individual patient's clinical situation allow guideline-based therapy aimed at maximizing surgical safety. [ABSTRACT FROM AUTHOR]