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Gynecologic Oncology Apr 2021To identify clinicopathological characteristics, treatment patterns, clinical outcomes and prognostic factors in patients with vulvar melanoma (VM).
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.
Topics: Aged; Cohort Studies; Disease-Free Survival; Female; Humans; Immune Checkpoint Inhibitors; Melanoma; Middle Aged; Molecular Targeted Therapy; Neoplasm Staging; Netherlands; Prognosis; Retrospective Studies; Survival Rate; United Kingdom; Vulvar Neoplasms
PubMed: 33514483
DOI: 10.1016/j.ygyno.2021.01.018 -
Bladder Cancer (Amsterdam, Netherlands) 2022Mucosal melanoma involving the urethra is a rare disease with distinct clinical and molecular characteristics and poor outcomes. Our current knowledge is limited by the...
BACKGROUND
Mucosal melanoma involving the urethra is a rare disease with distinct clinical and molecular characteristics and poor outcomes. Our current knowledge is limited by the small number of reports regarding this disease.
OBJECTIVE
To describe the clinical, pathological, and molecular characteristics of urethral melanoma.
METHODS
We summarized the clinicopathologic data for 31 patients treated for urethral melanoma from 1986-2017 at our institution. Genomic data from our institutional sequencing platform MSK-IMPACT ( = 5) and gene-specific PCR data on , , and/or ( = 8) were compared to genomic data of cutaneous melanomas ( = 143), vulvar/vaginal melanomas ( = 24), and primary non-melanoma urethral tumors ( = 5) from our institutional database.
RESULTS
Twenty-three patients were diagnosed with localized disease, 7 had regional/nodal involvement and one had metastases. Initial treatment included surgery in 25 patients; seven had multimodal treatment. Median follow-up was 46 months (IQR 33-123). Estimated 5-year cancer-specific survival was 45%. No significant change in survival was observed based on a year of treatment.Primary urethral melanomas showed a higher frequency of mutations compared to cutaneous (80.0% vs. 18.2%, = 0.006) and vulvar/vaginal melanomas (80.0 vs. 25.0%, = 0.04). mutations were absent in urethral primaries (0% vs. 46% in cutaneous melanoma, = 0.02). Tumor mutation burden was higher in cutaneous than urethral melanomas ( = 0.04). Urethral melanomas had a higher number of somatic alterations compared to non-melanoma urethral tumors (median 11 vs. 5, = 0.03).
CONCLUSIONS
Our findings support a unique mutational landscape of urethral melanoma compared to cutaneous melanoma. Survival remains poor and is unchanged over the time studied.
PubMed: 36277327
DOI: 10.3233/BLC-211633 -
Oncology (Williston Park, N.Y.) Jul 1996Melanomas of the vulva and vagina comprise < 2% of melanomas in women. Although their biologic behavior appears to be similar to that of cutaneous melanoma, vulvar and... (Review)
Review
Melanomas of the vulva and vagina comprise < 2% of melanomas in women. Although their biologic behavior appears to be similar to that of cutaneous melanoma, vulvar and vaginal melanomas appear to have a different etiology. Women presenting with pigmented vulvar lesions should undergo expedited examination and full-thickness biopsy. Vulvar and vaginal melanomas should be staged surgically using the AJCC system, which incorporates Breslow and Clark microstaging. Adverse prognostic factors include advanced age at diagnosis, central location of tumor, capillary lymphatic space involvement, ulceration, high mitotic rate, and aneuploidy. Primary surgery should include radical local excision with 1-cm skin margins for melanomas < 1 mm thick and 2-cm margins for melanomas 1 to 4 mm thick. Deep margins should be at least 1 to 2 cm. Femoral inguinal lymphadenectomy should be performed in patients at increased risk of lymph node metastases on the basis of primary tumor characteristics. Adjuvant interferon-alfa appears to confer survival benefits in patients with regional nodal disease. Effective salvage therapy has not yet been identified.
Topics: Age Factors; Aged; Aneuploidy; Biopsy; Female; Humans; Interferon-alpha; Lymph Node Excision; Lymphatic Metastasis; Lymphatic System; Melanoma; Mitosis; Neoplasm Staging; Prognosis; Salvage Therapy; Survival Rate; Ulcer; Vaginal Neoplasms; Vulvar Neoplasms
PubMed: 8837119
DOI: No ID Found -
Acta Obstetricia Et Gynecologica... Jun 2010No standard screening programs exist to detect vulvar carcinoma or its precursor lesions, and therefore gynecologists, dermatologists and other healthcare providers in... (Review)
Review
No standard screening programs exist to detect vulvar carcinoma or its precursor lesions, and therefore gynecologists, dermatologists and other healthcare providers in this field should be aware of the clinical features, behavior and management of the different existing premalignant vulvar lesions, squamous vulvar intraepithelial neoplasia (VIN), vulvar Paget's disease and melanoma in situ. In 2004, a new classification for squamous VIN was introduced by the International Society for the Study of Vulvar Disease, subdividing squamous VIN into the HPV-related usual type, and into differentiated type, which is associated with lichen sclerosus. This review describes the relevant aspects of squamous VIN, vulvar Paget's disease and melanoma in situ, its epidemiological characteristics, diagnosis, management and malignant potential.
Topics: Carcinoma in Situ; Epithelium; Female; Humans; Melanoma; Paget Disease, Extramammary; Precancerous Conditions; Skin Neoplasms; Vulvar Neoplasms
PubMed: 20504079
DOI: 10.3109/00016341003739575 -
Biomedicines Jun 2021Melanomas of the skin are poorly circumscribed lesions, very frequently asymptomatic but unfortunately with a continuous growing incidence. In this landscape, one can... (Review)
Review
Melanomas of the skin are poorly circumscribed lesions, very frequently asymptomatic but unfortunately with a continuous growing incidence. In this landscape, one can distinguish melanomas originating in the mucous membranes and located in areas not exposed to the sun, namely the vulvo-vaginal melanomas. By contrast with cutaneous melanomas, the incidence of these types of melanomas is constant, being diagnosed in females in their late sixties. While hairy skin and glabrous skin melanomas of the vulva account for 5% of all cancers located in the vulva, melanomas of the vagina and urethra are particularly rare conditions. The location in areas less accessible to periodic inspection determines their diagnosis in advanced stages, often metastatic. Moreover, despite the large number of drugs newly approved in recent decades for the treatment of cutaneous melanoma, especially in the category of biological drugs, the mortality of vulvo-vaginal melanomas has remained almost constant. This, together with the absence of specific treatment guidelines due to the lack of a sufficient number of cases to conduct randomized clinical trials, makes melanomas with this localization a discouraging diagnosis, associated with a very poor prognosis. Our aim is therefore to draw attention to this oftentimes overlooked entity in order to encourage the community to employ various strategies meant to increase research in this area. By highlighting the main risk factors of vulvar and vaginal melanomas, as well as the clinical manifestations and molecular changes underlying these neoplasms, ideally novel therapeutic schemes will, in time, be brought into effect.
PubMed: 34209084
DOI: 10.3390/biomedicines9070758 -
Sao Paulo Medical Journal = Revista... Jan 2010Vulvar melanoma is a rare disease. We describe the experience of a single institution and review the literature. (Review)
Review
CONTEXT AND OBJECTIVE
Vulvar melanoma is a rare disease. We describe the experience of a single institution and review the literature.
DESIGN AND SETTING
Retrospective study at the Department of Gynecology, Hospital do Cancer A. C. Camargo.
METHODS
Eleven patients with vulvar melanoma attended between January 1987 and December 2006 were reviewed regarding clinicopathological characteristics, surgical therapy and follow-up.
RESULTS
The initial symptoms were vulvar lesions, pruritus, pain and bleeding. The median age was 64.8 years. The median depth of invasion was 3.08 mm. The staging ranged from IB to IIIC (American Joint Committee on Cancer, 2002). All the patients underwent vulvectomy. Two patients did not undergo primary elective lymphadenectomy. Bilateral inguinal lymphadenectomy was performed on five patients, and one had unilateral inguinal lymphadenectomy. Sentinel lymph node investigation was performed on three patients. Five patients had locoregional recurrence. Prolonged survival was only achieved in the absence of lymph node involvement. The median follow-up was 56 months. The median disease-free survival was 15 months and the median overall survival was 29 months.
CONCLUSIONS
The prognosis for patients with vulvar melanoma is generally poor, with a high tendency towards regional and distant recurrence. Depth of invasion and lymph node involvement are the most important prognostic factors. In most cases, resection of the lesion with adequate margins may replace vulvectomy. Elective inguinal femoral lymphadenectomy remains the standard lymph node staging procedure. Sentinel lymph node investigation is feasible and should be performed by a multidisciplinary team with experience of this method.
Topics: Aged; Aged, 80 and over; Female; Follow-Up Studies; Humans; Lymph Node Excision; Melanoma; Middle Aged; Neoplasm Staging; Prognosis; Retrospective Studies; Sentinel Lymph Node Biopsy; Survival Rate; Vulvar Neoplasms
PubMed: 20512279
DOI: 10.1590/s1516-31802010000100008 -
Biomedicines Feb 2018vulvar intraepithelial neoplasia is a non-invasive precursor lesion found in 50-70% of patients affected by vulvar squamous cell carcinoma. In the past, radical surgery... (Review)
Review
BACKGROUND
vulvar intraepithelial neoplasia is a non-invasive precursor lesion found in 50-70% of patients affected by vulvar squamous cell carcinoma. In the past, radical surgery was the standard treatment for vulvar intraepithelial neoplasia, however, considering the psychological and physical morbidities related to extensive surgery, several less aggressive treatment modalities have been proposed since the late 1970s. Photodynamic therapy is an effective and safe treatment for cutaneous non-melanoma skin cancer, with favorable cosmetic outcomes.
METHODS
in the present paper, the results of selected studies on photodynamic therapy in the treatment of vulvar intraepithelial neoplasia are reported and discussed.
RESULTS
Overall, complete histological response rates ranged between 20% and 67% and symptom response rates ranged between 52% and 89% according to different studies and case series.
CONCLUSIONS
the real benefit of photodynamic therapy in the setting of vulvar intraepithelial neoplasia lies in its ability to treat multi-focal disease with minimal tissue destruction, preservation of vulvar anatomy and excellent cosmetic outcomes. These properties explain why photodynamic therapy is an attractive option for vulvar intraepithelial neoplasia treatment.
PubMed: 29393881
DOI: 10.3390/biomedicines6010013 -
Chinese Clinical Oncology Apr 2021Tracers and corresponding detection devices for the mapping of sentinel lymph nodes have been evolving since the first use of lymphangiogram methods in 1977 in penile... (Review)
Review
Tracers and corresponding detection devices for the mapping of sentinel lymph nodes have been evolving since the first use of lymphangiogram methods in 1977 in penile carcinoma. Nowadays a variety of dyes and radiotracers have been validated for use in breast, vulvar and cervical cancer as well as melanoma. Each tumor site with its anatomical conditions requires different mapping protocol. While the combination of radiotracer and blue dye or radiotracer alone is an established method for breast surgery, vulvar cancer and melanoma, in pelvic sentinel lymph node mapping indocyanine green is currently gaining popularity. Near infrared fluorescence imaging is an emerging technique that enables a real-time image-guided procedure and is currently approved by the Food and Drug Administration as a sentinel lymph node mapping substance with standard of care. New tracers and devices are constantly under investigation to better understand the pathway of lymphatic drainage and increase the sensibility and sensitivity of the method. In the present review the evolution of available tracers and detection devices is discussed. An exhaustive review of current clinical indications of each method, its particularities and adverse effects is made. Finally, an update on ongoing clinical studies in sentinel lymph node mapping methods is presented.
Topics: Colloids; Coloring Agents; Female; Humans; Lymph Nodes; Lymphatic Metastasis; Radiopharmaceuticals; Sentinel Lymph Node Biopsy; Technetium
PubMed: 33951916
DOI: 10.21037/cco-20-252 -
International Journal of Clinical and... 2012Primary mucosal melanomas arise from melanocytes located in mucosal membranes lining respiratory, gastrointestinal and urogenital tract. Although a majority of mucosal... (Comparative Study)
Comparative Study Review
Primary mucosal melanomas arise from melanocytes located in mucosal membranes lining respiratory, gastrointestinal and urogenital tract. Although a majority of mucosal melanomas originate from the mucosa of the nasal cavity and accessory sinuses, oral cavity, anorectum, vulva and vagina, they can arise in almost any part of mucosal membranes. Most of mucosal melanomas occur in occult sites, which together with the lack of early and specific signs contribute to late diagnosis, and poor prognosis. Because of their rareness the knowledge about their pathogenesis and risk factors is insufficient, and also there are not well established protocols for staging and treatment of mucosal melanomas. Surgery is the mainstay of treatment, with trends toward more conservative treatment since radical surgery did not show an advantage for survival. Radiotherapy can provide better local control in some locations, but did not show improvement in survival. There is no effective systemic therapy for these aggressive tumors. Compared with cutaneous and ocular melanoma, mucosal melanomas have lowest percent of five-year survival. Recently revealed molecular changes underlying mucosal melanomas offer new hope for development of more effective systemic therapy for mucosal melanomas. Herein we presented a comprehensive review of various locations of primary melanoma along mucosal membranes, their epidemiological and clinical features, and treatment options. We also gave a short comparison of some characteristics of cutaneous and mucosal melanomas.
Topics: Combined Modality Therapy; Female; Genital Neoplasms, Female; Head and Neck Neoplasms; Humans; Male; Melanocytes; Melanoma; Mouth Neoplasms; Mucous Membrane; Paranasal Sinus Neoplasms; Rectal Neoplasms; Skin Neoplasms; Survival Rate
PubMed: 23071856
DOI: No ID Found -
Current Oncology (Toronto, Ont.) Apr 2022In order to identify the molecular pathways governing melanoma and track its progression, the next-generation sequencing (NGS) approach and targeted sequencing of cancer... (Review)
Review
In order to identify the molecular pathways governing melanoma and track its progression, the next-generation sequencing (NGS) approach and targeted sequencing of cancer genes were employed. The primary tumor, as well as metastatic tissue, of an 84-year-old patient diagnosed with vulvar melanoma (VM), were investigated. The primary tumor specimen showed multiple somatic mutations in gene, suggesting its major contribution to melanoma origin. The metastatic sample showed additional alterations, including other melanoma-related genes. Clinical relevancy is postulated to juxtamembrane region instability of gene (c-KIT). We did not identify or alterations, which are typical for the most common melanoma pathway-MAPK cascade. However, it should be noted that this is the first report evidencing in melanoma, although its role in triggering VM needs to be further elucidated.
Topics: Aged, 80 and over; Humans; MAP Kinase Signaling System; Melanoma; Mutation; Proto-Oncogene Proteins B-raf; Skin Neoplasms
PubMed: 35621644
DOI: 10.3390/curroncol29050254