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Vulvar and vaginal melanomas (VVMs) are rare and aggressive malignancies with limited prognostic models available and there is no standard reporting protocol. VVMs were selected from six tertiary Canadian hospitals from 2000-2021, resected from patients aged ≥18 years, with 6 months or longer follow-up data, and confirmation of melanocytic differentiation by at least two immunohistochemical markers. Cases were reviewed by pathologists to identify histological biomarkers. Survival outcomes were tested with Kaplan-Meier log-rank, univariate Cox, and multivariate Cox regression. There were 79 VVMs with median follow-up at 26 months. Univariate analysis revealed that tumour necrosis, tumour ulceration, positive lymph nodes, and metastasis at diagnosis were significantly associated with disease-specific mortality, progression, and metastasis. Multivariate analysis identified tumour necrosis as an independent prognostic factor for disease-specific mortality (HR 4.803, 95% CI 1.954-11.803, p<0.001), progression (HR 2.676, 95% CI 1.403-5.102, p=0.003), and time-to-metastasis for non-metastatic patients at diagnosis (HR 3.761, 95%CI 1.678-8.431, p=0.001). Kaplan-Meier survival analyses demonstrated that tumour necrosis was a poor prognostic factor for disease-specific, progression-free, and metastasis-free survival (p<0.001 for all comparisons). Vaginal melanomas displayed decreased survival compared to vulvar or clitoral melanomas. This study identifies tumour necrosis as an independent prognostic factor for VVMs. Vaginal melanomas specifically showed worse survival outcomes compared to vulvar or clitoral melanomas, consistent with previously reported findings in the literature, emphasising the importance of differentiating between these primary tumour epicentres for prognostication and treatment planning in the care of genital melanoma patients., (Copyright © 2024 Royal College of Pathologists of Australasia. Published by Elsevier B.V. All rights reserved.)

Background: Vulvar and vaginal melanoma (VuM & VaM) is a rare gynecologic malignancy with high mortality but low effectiveness to checkpoint immunotherapy compared to cutaneous melanoma. This article aims to elucidate the role of the disordered immune microenvironment in cancer progression in VuM., Methods: At first, this article applied single-cell RNA sequencing (scRNA-seq) to the VuM obtained from a 68-year-old female patient, and constructed a single-cell atlas of VuM consist of 12,243 single cells. Then this article explores the genomic complexity and core signal channel in VuM microenvironment., Results: This article provides new insights about the pathogenesis of VuM based on single-cell resolution data. It was found that the activation of CD8 + T cell contributed to induce tumor angiogenesis and immune escape, and the activation of the antigen-presenting molecular function participated in melanoma metastasis., Conclusion: This article provided new insights into underlining VuM molecular regulation and potential signaling involved in immunotherapy, which would benefit the clinical practice and administration., (© 2024. The Author(s).)

Objective: This multicenter study aimed to investigate the role of preoperative lymphatic mapping and sentinel node biopsy (SNB) as well as the impact of negative SNB on loco-regional control and survival in vulvar melanoma patients with clinically negative nodes (cN0)., Methods: Patients who had a proven vulvar melanoma with a Breslow thickness of 1-4 mm, cN0 and underwent a preoperative lymphatic mapping followed by SNB between July 2013 and March 2021 were retrospectively included. Groin recurrence and mortality rate were calculated as absolute and relative frequency. Disease-free survival (DFS) and overall survival (OS) were assessed by the Kaplan-Meier method. We provided a systematic review, searching among PubMed/Medline and Embase libraries. A total of 6 studies were identified (48 patients)., Results: A total of 18 women were included. Preoperative planar images showed 51 SNs in 28 groins. Additional SPECT/CT images were acquired in 5/18 cases; SNs were identified pre- and intra-operatively in all cases. A total of 65 SNs were excised from 28 groins. A total of 13/18 (72.2%) patients (21/28 groins, 75%) had negative SNs with no groin recurrences and 12/13 (92.3%) were still alive at last follow-up. Five out of the 18 (27.8%) patients (7/28 groins, 25%) had positive SNs, 2/5 (40%) patients died of cancer after 26.2 and 33.8 months, respectively. The median DFS and OS for the entire cohort were 17.9 months (95% CI, 10.3-19.9) and 65.0 months (95% CI, 26.2-infinite), respectively. The probability of DFS and OS at 3 years were 15.5% (95% CI, 2.6-38.7) and 64.3% (95% CI, 15.5-90.2), respectively., Conclusions: The use of preoperative lymphatic mapping followed by SNB permits a precise and minimally invasive surgical approach in cN0 vulvar melanoma patients. Negative SNB is associated with low risk of groin relapse and good survival., Competing Interests: Declaration of Competing Interest The authors declare no conflict of interest., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Female genital tract melanoma (FGTM) is a rare and aggressive melanocytic malignancy, and its clinico-pathological and prognostic features at different anatomic sites have not yet been fully described. We retrospectively analyzed and compared the clinico-pathological data and survival outcomes of patients with primary lower genital tract melanoma enrolled between January 2005 and December 2020. We identified 95 patients with FGTM, of whom 46 had vulvar melanomas (VuM), 43 had vaginal melanomas (VaM), and six had cervical melanomas (CM). The clinical characteristics of all 95 cases, including symptoms, single or multiple primary lesions, clinical stage, surgery, and histopathological characteristics of 62 primary untreated cases, including pigmentation, predominant cytology, histological pattern, mitotic figures, and tumor-infiltrating lymphocytes of VuM, VaM, and CM, differed significantly. In comparison, only trend differences in molecular alternations were evident (p = 0.077). Disease-specific survival (DSS) was 30.7% at 5 years (46.5%, 25.6%, and 44.4% for VuM, VaM and CM, respectively). Seventy-one (85.5%) patients experienced FGTM recurrence. The median time to the first recurrence was 11 months, and VaM recurred earlier than VM and CM (16, 6, and 10 months for VuM, VaM, and CM, respectively, p = 0.038). A univariate analysis of 50 cases revealed the negative factors of disease-specific survival (DSS), including the location of the vagina and the presence of ulceration, and the negative factors of recurrence-free survival (RFS), including multiple lesions, the presence of ulceration, and the presence of lymphovascular invasion. Multiple lesions showed a borderline correlation with DSS. A multivariate Cox regression analyses of 50 cases revealed that the presence of ulceration was associated with shorter DSS and RFS (yes vs. no, Hazard Ratio = 2.400 and 2.716, respectively). Vaginal location showed a significant correlation with DSS (Hazard Ratio = 2.750, p = 0.024). In conclusion, vulval, vaginal, and cervical melanomas may differ in terms of their clinico-pathological features and associations with DSS and RFS. Ulceration and vaginal location were significantly associated with shorter DSS, and ulceration was associated with an increased risk of FGTM recurrence., (Copyright © 2022. Published by Elsevier Inc.)

Vulvar melanoma is a rare tumour, of poorly known pathogenesis, which presents particularities compared to cutaneous melanoma: more frequently achromic and very often multifocal, it is of the lentiginous histological type (MLM), with in half of the cases lentiginous melanocytic hyperplasia at a distance., (Copyright © 2020 Elsevier Masson SAS. All rights reserved.)

Objective: To identify clinicopathological characteristics, treatment patterns, clinical outcomes and prognostic factors in patients with vulvar melanoma (VM)., Materials & Methods: This retrospective multicentre cohort study included 198 women with VM treated in eight cancer centres in the Netherlands and UK between 1990 and 2017. Clinicopathological features, treatment, recurrence, and survival data were collected. Overall and recurrence-free survival was estimated with the Kaplan-Meier method. Prognostic parameters were identified with multivariable Cox regression analysis., Results: The majority of patients (75.8%) had localized disease at diagnosis. VM was significantly associated with high-risk clinicopathological features, including age, tumour thickness, ulceration, positive resection margins and involved lymph nodes. Overall survival was 48% (95% CI 40-56%) and 31% (95% CI 23-39%) after 2 and 5 years respectively and did not improve in patients diagnosed after 2010 compared to patients diagnosed between 1990 and 2009. Recurrence occurred in 66.7% of patients, of which two-third was non-local. In multivariable analysis, age and tumour size were independent prognostic factors for worse survival. Prognostic factors for recurrence were tumour size and tumour type. Only the minority of patients were treated with immuno- or targeted therapy., Conclusion: Our results show that even clinically early-stage VM is an aggressive disease associated with poor clinical outcome due to distant metastases. Further investigation into the genomic landscape and the immune microenvironment in VM may pave the way to novel therapies to improve clinical outcomes in these aggressive tumours. Clinical trials with immunotherapy or targeted therapy in patients with high-risk, advanced or metastatic disease are highly needed., Competing Interests: Declaration of Competing Interest The authors declare that they have no conflict of interest. Authors have full control of all primary data. They agree to allow the journal to review the data if requested., (Copyright © 2021 The Authors. Published by Elsevier Inc. All rights reserved.)

Vulvar cancer (VC) is a rare neoplasm, usually arising in postmenopausal women, although human papilloma virus (HPV)-associated VC usually develop in younger women. Incidences of VCs are rising in many countries. Surgery is the cornerstone of early-stage VC management, whereas therapies for advanced VC are multimodal and not standardized, combining chemotherapy and radiotherapy to avoid exenterative surgery. Randomized controlled trials (RCTs) are scarce due to the rarity of the disease and prognosis has not improved. Hence, new therapies are needed to improve the outcomes of these patients. In recent years, improved knowledge regarding the crosstalk between neoplastic and tumor cells has allowed researchers to develop a novel therapeutic approach exploiting these molecular interactions. Both the innate and adaptive immune systems play a key role in anti-tumor immunesurveillance. Immune checkpoint inhibitors (ICIs) have demonstrated efficacy in multiple tumor types, improving survival rates and disease outcomes. In some gynecologic cancers (e.g., cervical cancer), many studies are showing promising results and a growing interest is emerging about the potential use of ICIs in VC. The aim of this manuscript is to summarize the latest developments in the field of VC immunoncology, to present the role of state-of-the-art ICIs in VC management and to discuss new potential immunotherapeutic approaches.

Background: Primary vulvar melanoma was an aggressive and poorly understood gynecological tumor. Unlike cutaneous melanoma, the incidence of vulvar melanoma was low but the survival was poor. There were no standard staging system and no census on treatment strategies of vulvar melanoma. Therefore, we aimed to conduct and validate a comprehensive prognostic model for predicting overall survival of vulvar melanoma and provide guidance for clinical management., Methods: Patients diagnosed with vulvar melanoma between year 2004 and 2015 from Surveillance, Epidemiology, and End Result (SEER) database were randomized to training cohort and validation cohort. Multivariate survival analysis was performed to screen for independent factors of survival. A nomogram was established to predict overall survival of vulvar melanoma. Receiver operating characteristic curve and calibration plot were performed to verify the discrimination and accuracy of the model. The decision curve analysis was performed to verify the clinical applicability of the model., Results: Total 737 patients with vulvar melanoma were randomized to the training cohort (n = 517) and the validation cohort (n = 220). Nomogram including age, race, tumor site, depth of tumor invasion, lymph node status, distant metastasis, tumor size, surgery, chemotherapy and radiotherapy was established and validated. The c-indexes for SEER stage, American Joint Committee on Cancer stage and this model were 0.561, 0.635 and 0.826, respectively. The high-risk group scored by this model had worse survival than the low-risk group (P < 0.001). Decision curve analysis revealed this model was superior in predicting survival., Conclusions: Our model was deemed to be a useful tool for predicting overall survival of vulvar melanoma with good discrimination and clinical applicability. We hoped this model would assist gynecologists in clinical decision and management of patients diagnosed with vulvar melanoma., (© The Author(s) 2020. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permission@oup.com.)

An atypical cellular blue nevus, a benign mass, may sometimes transform into a malignant melanoma. Here, we report a rare case of melanoma arising in a large congenital vulvar blue nevus. A 28-year-old Chinese woman presented to our hospital with a chief complaint of a vulvar mass that had persisted for 8 years. The patient underwent extensive local excision, followed by reconstructive surgery of the female reproductive tract. The mass was diagnosed as being a vulvar malignant melanoma. Postoperatively, the patient received interferon immunotherapy and recovered without complications. No evidence of recurrence was observed after 32 months of follow-up. Our case thus shows that comprehensive treatment with surgery supplemented by immunotherapy can be effective against a malignant melanoma arising in a vulvar blue nevus., (© 2020 Wiley Periodicals LLC.)

Introduction: Mucosal melanoma is rare and associated with poorer prognosis in comparison to conventional melanoma subtypes. Little is known about the prognostic significance as well as possible associations between PARP1 and immunologic response in mucosal melanoma., Methods: PARP1, PD-L1 and IDO1 immunostains were performed on 192 mucosal melanomas including 86 vulvar, 89 sinonasal, and 17 anorectal melanomas., Results: By Kaplan-Meier analyses, high PARP1 expression correlated with worse overall and melanoma-specific survival (log-rank p values = 0.026 and 0.047, respectively). Tumors with combined PARP1 and IDO1 high expression correlated with worse overall and melanoma-specific survival ( p = 0.015, 0.0034 respectively). By multivariate analyses, high PARP1 expression remained a predictor of worse survival independent of stage. By Fisher's exact test, high PARP1 expression correlated with highly mitogenic tumors ( p = 0.02). High tumoral PD-L1 and IDO1 expression were associated with ulcerated primary tumors ( p = 0.019, 0.0019, respectively). By linear regression analyses, correlations between PARP1 expression versus IDO1 expression ( p = 0.0001) and mitotic index ( p = 0.0052) were observed., Conclusion: Increased expression of PARP1 is an independent negative prognostic marker in mucosal melanomas. The association between PARP1 and IDO1 and their combined adverse prognostic role raise the potential of combined therapy in mucosal melanoma.

Objective: This study sought to evaluate the clinicopathologic features, surgical management, and survival of patients over 12 years at two academic centres., Methods: Patients diagnosed with vulvar or vaginal melanoma between 2002 and 2014 were identified through pathology databases. Clinical and pathologic data were extracted from the medical records. The Kaplan-Meier method was used to calculate recurrence-free survival and overall survival (OS), and univariate analyses using a Cox proportional hazard model were used to detect covariates related to survival., Results: Patients with vulvar melanoma were more likely to undergo surgical excision (84.0% vs. 55.6%, P = 0.0243) and were more likely to achieve negative margins (70.0% vs. 16.7%, P < 0.0001). Forty-eight percent of patients with vulvar melanoma had a lymph node evaluation; sentinel node biopsies were performed in 32%. Actuarial median OS for vulvar melanoma was 45 months compared with 10.48 months for vaginal melanoma. A subset of 10 patients with vulvar melanoma who survived longer than 60 months was identified. Eight significant predictors of OS were demonstrated for vulvar melanomas: clinical stage, maximum tumour size, tumour thickness, lymphovascular space invasion status, clinically enlarged lymph nodes, sentinel lymph nodes, lymph node status, and radiation treatment. Patients with positive or indeterminate margin status demonstrated a higher risk of recurrence than did patients with negative margins (hazard ratio 2.60; 95% CI 1.14-5.90)., Conclusion: Surgical excision with adequate margins is the mainstay of primary management when feasible. Lymph node evaluation, including sentinel nodes, may be considered in selected patients. Vulvar and vaginal sites differ markedly with respect to pathology, initial management, and survival, and they should be evaluated separately., (Copyright © 2018 The Society of Obstetricians and Gynaecologists of Canada/La Société des obstétriciens et gynécologues du Canada. Published by Elsevier Inc. All rights reserved.)

Background: Although the most commonly recommended treatment for melanoma and extramammary Paget's disease (EMPD) of the genital region is wide surgical excision of the lesion, the procedure is highly invasive and can lead to functional and sexual problems. Alternative treatments have been used for local control when wide local excision was not feasible. Here, we describe four patients with genital malignancies who were treated with boron neutron capture therapy (BNCT)., Methods: The four patients included one patient with vulvar melanoma (VM) and three with genital EMPD. They underwent BNCT at the Kyoto University Research Reactor between 2005 and 2014 using para-boronophenylalanine as the boron delivery agent. They were irradiated with an epithermal neutron beam between the curative tumor dose and the tolerable skin/mucosal doses., Results: All patients showed similar tumor and normal tissue responses following BNCT and achieved complete responses within 6 months. The most severe normal tissue response was moderate skin erosion during the first 2 months, which diminished gradually thereafter. Dysuria or contact pain persisted for 2 months and resolved completely by 4 months., Conclusions: Treating VM and EMPD with BNCT resulted in complete local tumor control. Based on our clinical experience, we conclude that BNCT is a promising treatment for primary VM and EMPD of the genital region. Trial registration numbers UMIN000005124.

Background/aim: Orbital metastasis of systemic cancer is exceedingly rare. This is a case report of a patient treated for locally recurrent vulvar melanoma who later presented with unilateral proptosis and was found to have an isolated biopsy-proven extraocular muscle metastasis., Patients and Methods: A 94-year-old female with locally recurrent vulvar melanoma presented with eye discomfort and blurry vision. Patient underwent histopathological, genetic, and imaging studies., Results: All prior work-up, including brain MRI and PET/CT, was negative for disease elsewhere from local recurrence. Orbital MRI demonstrated a mass involving the extraocular muscle, and immunohistochemistry staining of biopsy was consistent with metastasis. The patient underwent radiation therapy and tolerated treatment well., Conclusion: This is the first reported case of vulvar melanoma with extraocular muscle metastasis. The absence of findings on imaging as part of the staging work-up underscores the importance of considering extraocular muscle (EOM) metastasis as a differential for patients with vulvar melanoma who present with proptosis., (Copyright© 2018, International Institute of Anticancer Research (Dr. George J. Delinasios), All rights reserved.)

The prognostic role of PDL1 expression, CD8+ and FoxP3+ lymphocytes in vulvar melanomas has not been studied. We correlated PDL1 expression and CD8+ and FoxP3+ immune infiltrates with clinicopathologic variables and patient outcomes in a series of 75 vulvar melanomas. Tumoral PDL1 expression (>5%) was seen in 23% of cases. By Fisher exact test, PDL1 expression and peritumoral FoxP3+ lymphocytes significantly correlated with less disease-specific death. By linear regression analysis, correlations between tumoral PDL1 expression with the density of tumoral CD8+ and peritumoral CD8+ lymphocytes, tumoral FoxP3+ with tumoral CD8+ lymphocytes, and peritumoral FoxP3+ with peritumoral CD8+ lymphocytes were observed. By univariate analyses, tumor thickness >4 mm predicted poorer progression-free survival, melanoma-specific survival, and overall survival. PDL1 expression >5% and peritumoral CD8+, peritumoral FoxP3+, and tumoral FoxP3+ lymphocytes correlated with better overall survival. By multivariate analyses, high peritumoral FoxP3+ lymphocytes independently predicted better melanoma-specific survival (P = .023), and tumor thickness independently predicted poorer progression-free survival (P = .05) and overall survival (P = .039). In conclusion, our study shows that, independent from tumor thickness, an increased density of peritumoral FoxP3+ lymphocytes may positively impact survival in a subset of vulvar melanomas. Tumoral PDL1 expression correlated with tumoral as well as peritumoral CD8+ and FoxP3+ lymphocytes, supportive of an adaptive immune response. Although the frequency of PDL1 expression is low in vulvar melanoma, its expression may identify a subset of vulvar melanoma that might respond to immunotherapy., (Copyright © 2018. Published by Elsevier Inc.)

Background: Genital melanosis may clinically mimic melanoma. Little is known about the potential risk for genital and nongenital melanoma in these patients., Objective: In this retrospective cohort study, we analyzed clinical and histologic data from patients with genital melanosis to better characterize these lesions and the risk they confer for genital and nongenital melanoma., Methods: In all, 41 patients were identified for a retrospective chart review and histologic analysis., Results: Genital melanosis can clinically mimic melanoma but the typical age of onset is younger than for genital melanoma. A majority of lesions were found to stabilize or regress over time. Five patients were found to have a history of melanoma, only 1 of which was in the genital region. Lesions from these patients were more likely to show melanocytes with suprabasal movement (P = .0101) and to have a higher melanocyte count (P < .0462)., Limitations: We present a relatively small cohort of patients with an average follow-up of only 30.5 months., Conclusion: Patients with genital melanosis, and in particular those with any level of histologic atypia in the genital melanosis lesion, may require careful total body skin examinations for the possibility of melanoma in any body site., (Copyright © 2016 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.)

Background: Optimal treatments for vulvar and vaginal melanomas (VVMs) have not been identified. Herein, the authors compare molecular profiles between VVM and nongynecologic melanoma (NGM) subtypes with the objective of identifying novel, targetable biomarkers., Methods: In total, 2304 samples of malignant melanoma that were submitted to Caris Life Sciences between 2009 and 2015 were reviewed. In situ hybridization and immunohistochemistry were used to assess copy numbers and protein expression of selected genes. Sequenced variants were analyzed using a proprietary cancer panel., Results: In total, 51 VVMs (14 vaginal and 37 vulvar melanomas) were compared with 2253 malignant NGMs, including 2127 cutaneous, 105 mucosal, and 21 acral melanomas. In VVMs, B-Raf proto-oncogene serine/threonine kinase (BRAF) was the most frequently mutated gene (26%) compared with 8.3% of mucosal NGMs (P = .008). In BRAF-mutated tumors, fewer VVMs (50%), compared with NGMs (82.1%), had a variant within the valine codon 600 (V600) domain. The KIT mutation rate was highest in VVMs (22%) compared with 3% in cutaneous (P < .001) and 8.8% in mucosal (P = .05) melanoma subtypes. NRAS mutations were rare in VVMs compared with cutaneous (25.9%; P = .009) and acral (40.6%; P = .002) melanoma subtypes. PD-L1 (56%) and PD-1 (75%) were frequently expressed in VVM, whereas PI3KCA pathway mutations and estrogen receptor/progesterone receptor expression were rare. Compared with VVMs that had KIT mutations, wild-type KIT VVMs were more likely to express molecular markers suggestive of platinum resistance (ERCC1), alkylating sensitivity (MGMT), and anthracycline sensitivity (TOP2A)., Conclusions: The unique molecular features of VVM render this disease a distinct subtype of melanoma. Gene-based molecular therapy and immunotherapies may be promising and should be evaluated in clinical trials. Cancer 2017;123:1333-1344. © 2016 American Cancer Society., (© 2016 American Cancer Society.)

Objective: To describe trends in incidence, treatment and survival of patients with basal cell carcinomas and melanomas of the vulva. Also to compare survival of vulvar and cutaneous melanoma patients., Methods: All women with a vulvar malignancy between 1989 and 2012 were selected from the Dutch Cancer Registry (n=6436). Standardized incidence rates, estimated annual percentage change (EAPC) and 5-year relative survival rates were calculated for basal cell carcinomas (BCCs) and melanomas. Patients with vulvar melanomas were matched to women with cutaneous melanomas on period of diagnosis, age, Breslow thickness, tumour ulceration, lymph node status and distant metastases. Differences in survival were evaluated using Kaplan-Meier curves and the log rank test., Results: 489 women were diagnosed with a BCC and 350 with a melanoma of the vulva. The EAPC in incidence for melanomas was 0.2% and 1.1% for BCCs. Eighty-six percent of patients with BCC underwent surgical treatment in 1989-2006 and 95% in 2005-2012. Forty-five percent with BCC and 79% with melanoma were treated in a referral centre. Five-year relative survival for BCCs was 100% and for melanomas survival increased from 37% (95%CI 28-47%) in 1989-1999 to 45% (95%CI: 37-54%) in 2000-2012. Five years after diagnosis survival of women with vulvar melanoma was 15% lower compared to matched cutaneous melanoma patients (p=0.002)., Conclusion: No trends in age-adjusted incidence have been observed but more patients with BCC received surgical treatment over time. Having had vulvar BCC did not affect life expectancy. Well-known prognostic factors explained most of the differences in survival between cutaneous and vulvar melanoma patients, however a difference of 15% remained unexplained., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Although rare, vulvar melanoma is the second most common histological type of vulvar cancer, representing around 10% of all malignant tumors involving the vulva presenting typically in post-menopausal women. The data on vulvar melanoma consists almost entirely of case studies and small retrospective series. A standardized approach to the diagnosis and management of vulvar melanoma is not available. In this paper, a case of vulvar melanoma in remission is described and a review of the current literature is presented. A 73-year-old, G5P5 (5-0-0-5), complained of a 3-year history of intense vaginal pruritus. A biopsy was done at the site of the hyperpigmented patch revealing vulvar melanoma. CT scan was done before surgery and revealed negative lymph node invasion nor metastasis. The patient underwent a radical vulvectomy followed by adjuvant radiotherapy which offered remission of disease for 5 years. [ABSTRACT FROM AUTHOR]

Simple Summary: Vulvar melanoma (VM) is a rare and fatal disease. Knowledge of this disease is poor and, consequently, so is knowledge of the prognosis. Our aim was to collect evidence on VM with particular attention to its molecular profile and related clinical and therapeutic implications. Malignant melanoma is a fatal disease that affects all skin sites. Among these, vulvar melanoma (VM) is a rare gynecological condition that accounts for 5% of all vulvar neoplasms. VM primarily affects older Caucasian women and its relationship to sun exposure is undefined. Diagnosis is defined by biopsy but many clinical, dermatoscopic, and confocal microscopic features can guide doctors. The molecular profile is characterized by the KIT mutation, revealed by all of the technologies that are used (classical sequencing, next-generation sequencing, and immunohistochemical staining). BRAF and NRAS mutations are also common in VM. All of these mutations are possible therapeutic targets. Today, surgery remains the first treatment choice for primary VM. The role of neoadjuvant and adjuvant therapy is scarce and the treatment of relapses is widely debated. [ABSTRACT FROM AUTHOR]

Vulvar malignant melanoma (VMM) is uncommon and poses a significant management challenge. Here, we presented a case of VMM managed by surgery, chemoradiation, and planned for targeted therapy. A 70-year-old woman with underlying diabetes mellitus and hypertension presented with a black-colored exophytic growth around her left vulva for two months. Initial biopsy confirmed malignant melanoma with positive staining for S100, HMB 45, and Melan A. An imaging study showed that the disease was localized to the vulva. She underwent bilateral radical vulvectomy and bilateral inguinofemoral lymph node dissection followed by radiotherapy. She had a locoregional disease recurrence, which was subsequently managed by palliative perineal radiotherapy, chemotherapy, and planned for immunotherapy. Vulvar malignant melanoma is a rare and aggressive tumor, with a poor overall prognosis, and high recurrence rate. Adjuvant chemotherapy, radiotherapy, and immunotherapy may be beneficial for local recurrence and distant metastasis cases. Molecular Analysis has a potential role in targeted therapy to improve the survival and outcome of the patient. [ABSTRACT FROM AUTHOR]

Introduction. Malignant skin melanoma accounts for 1% of all human malignancies. Vulvar melanoma constitutes less than 1% of all melanomas and it is the second most common malignant tumour of the vulva, accounting for 10% of all vulvar malignancies. Usually, it is detected in a later stage compared to other skin melanomas because of its anatomical location and represents a real diagnostic and treatment challenge for dermatologists and gynaecologists. Case presentation. We present a rare clinical case of a 27-year-old woman with pigmented malignant melanoma affecting the left labium majora. Conclusions. Malignant vulvar melanoma in women under the age of 45 years is rare. It is more aggressive than other types of malignant skin melanomas because of its relatively late diagnosis. Knowledge of the disease will improve the medical care provided and patients’ prognosis.

Vulvar malignant melanoma is the second most common subtype of vulvar cancer, accounting for 5–10% of all vulvar cancers. The prognosis is still very poor, although some advances have been achieved in the last years. One of the most significant changes in its management has been the development of less invasive surgical techniques that diminish the risk of postoperative morbidity and long-lasting sequelae. In this article, we review the surgical management of the pathology, based on the comment of 3 cases with vulvar melanoma treated at our institution. [ABSTRACT FROM AUTHOR]

Vulvar melanoma is a rare and deadly cancer in women, and the prognosis is often poor. There are limited studies on the dermoscopic features of vulvar melanoma. Described criteria include the presence of blue, gray, or white colors. Herein we present the clinical and dermoscopic characteristics of a hypopigmented and heavily pigmented nodule in a 92-year-old and an 80-year-old woman. Dermoscopy in the former revealed structureless milky-red to white areas, remnants of brown pigmentation at the base and polymorphic vessels, while the latter displayed structureless blue-gray areas with black dots and peripheral lines at the base. In both cases, histopathology revealed a stage III melanoma. Our two cases along with a review of the literature suggest that the dermoscopic features described for diagnosing cutaneous nodular melanoma, apply also for vulvar melanoma. Clinicians should always raise the suspicion if observing plaques or nodules with a dermoscopic polymorphic vascular pattern and blue-black color on the genitals of postmenopausal women.

Aim of the study: In this retrospective cohort study we have examined differences in survival profiles with respect to the body mass index in patients with mucosal melanoma on immune checkpoint inhibitor therapy. Materials and methods: The primary outcome included the association between the body mass index and overall survival in patients with metastatic mucosal melanoma. The secondary outcomes included the clinical presentation and management of vulvar and vaginal melanomas with oral and anorectal mucosal melanomas, as well as the surgical and radiological management of vulvar and vaginal melanomas. Kaplan--Meier analysis and log-rank test were used for the assessment of overall survival. Results: The results showed that patients with mucosal melanoma whose body mass index was ≥25 had better overall survival (p = 0.02). Overall survival was different between vulvar/vaginal vs. oral mucosal melanoma (p = 0.02). Overall survival was not different between vulvar/vaginal vs. anorectal melanoma (p = 0.77). Some immune toxicities were specific to patients with vulvar/vaginal melanoma. Conclusions: Obesity is associated with improved survival in patients with metastatic mucosal melanoma, although findings can be heterogeneous depending on the subtype of mucosal melanoma. [ABSTRACT FROM AUTHOR]

The soft tissue defects of the anterior perineum can be caused by tumour excision, trauma, burns, Fournier's gangrene and genital mutilation. Various reconstruction methods were described: vacuum-assisted closure, skin grafts and skin substitutes, pedicled or free flaps. In the case of defects following oncologic surgery, flaps are the reconstructive method of choice. We present the case of a 65 year-old woman diagnosed with stage III A vulvovaginal melanoma affecting also the distal urethra. Radical vulvectomy and en-bloc excision of the anterior third of the vagina and distal urethra, along with bilateral inguinal lymphadenectomy was performed. The resulting defect was covered with bilateral V-Y advancement gluteal fold flaps. A local vaginal recurrence appeared 18 months after surgery and was safely removed. The patient followed chemotherapy with Dacarbazine. The gluteal fold flap offered good defect coverage and survived completely. Follow-up period was two years without distant metastases. Functional result was excellent, urinary function being normal and patient satisfaction high. Due to the gluteal flap's advantages (appropriate thickness, good vascularity, minimal donor site morbidity, and same operative field allowing for shorter operating time), reconstruction of the anterior perineum after oncological resection with bilateral V-Y advancement gluteal flaps is a viable solution. [ABSTRACT FROM AUTHOR]