UNASSIGNED: Vulvar melanoma and vaginal melanoma are rare and difficult to treat. We describe the last three decades in a cohort predominantly treated surgically with adjuvant therapy.
UNASSIGNED: All new patients between 1993 and 2021 followed until 2024. Collection of demographic and oncologic data allowed comparisons and Kaplan-Meier method was used to evaluate overall survival (OS) and progression free survival (PFS) stratified by adjuvant therapy type, diagnosis before and after 2011, and between vulvar and vaginal melanomas.
UNASSIGNED: Consultation for 63/72 patients (87.5 %) were for primary treatment. Most patients had vulvar melanoma (50/72, 69.4 %), received surgery (65/72, 90.3 %), and adjuvant treatment (40/72, 55.6 %) with immunotherapy, chemotherapy, and/or targeted therapy. Median survival for 63 patients presenting for primary treatment was 54.2 months, and 9/13 patients who were disease free after five years later received adjuvant immunotherapy. Survival did not vary by adjuvant therapy type or diagnosis after 2011 but was significantly less for vaginal melanoma. Following recurrence seven patients experienced complete response including three patients receiving combined nivolumab with ipilimumab and two nivolumab alone experienced.
UNASSIGNED: Survival was not significantly different by adjuvant therapy type or after 2011. Most patients who were disease-free five years after surgery had received adjuvant therapy. Seven patients experienced complete responses to therapy after recurrence of whom five received immune checkpoint inhibitors. Although survival is not improved as in cutaneous melanomas by immune checkpoint inhibitors, signal continues for the use of immune checkpoint inhibitors in gynecologic melanomas.

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.

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.
CONCLUSIONS: This article provided new insights into underlining VuM molecular regulation and potential signaling involved in immunotherapy, which would benefit the clinical practice and administration.

The incidence of vulvar carcinoma increases with age, though elderly women receive less aggressive cancer therapies and fewer strategies aimed at cancer prevention. Furthermore, elderly women dual enrolled in Medicaid-Medicare experience poor survival rates for vulvar carcinoma. Herein, we provide recommendations for the prevention of and guidelines for the multidisciplinary care of vulvar carcinoma. Prevention of vulvar carcinoma can be categorized into primary, secondary, and tertiary prevention. Primary prevention consists of vaccination, secondary prevention consists of screening, and tertiary prevention is aimed at the management of premalignant and early-stage lesions.

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).
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).
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.
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.

暂无摘要。

The aim of this article is to describe an island flap, harvested from the inguinal fold, which can be used for vulvar reconstruction: the inguinal fold island flap (IFI flap). IFI flap is indicated for reconstruction of defects of vaginal vestibule and labia minora and it could be raised bilaterally safeguarding regional symmetry and avoiding vaginal introitus or urethral distortion. This flap has been utilized to reconstruct defects after vulvar melanoma and squamous cell carcinoma resections and in one case to restore vaginal vestibule anatomy in a revision surgery in a transgender woman. IFI flap is an example of an \"aesthetic/functional\" reconstruction which could be proposed to younger patients too.

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.

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.

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.