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Biomedicines Jan 2022Mucosal melanomas (MM) are rare tumors, being less than 2% of all diagnosed melanomas, comprising a variegated group of malignancies arising from melanocytes in... (Review)
Review
Mucosal melanomas (MM) are rare tumors, being less than 2% of all diagnosed melanomas, comprising a variegated group of malignancies arising from melanocytes in virtually all mucosal epithelia, even if more frequently found in oral and sino-nasal cavities, ano-rectum and female genitalia (vulva and vagina). To date, there is no consensus about the optimal management strategy of MM. Furthermore, the clinical rationale of molecular tumor characterization regarding BRAF, KIT or NRAS, as well as the therapeutic value of immunotherapy, chemotherapy and targeted therapy, has not yet been deeply explored and clearly established in MM. In this overview, focused on anorectal and genital MM as models of rare melanomas deserving of a multidisciplinary approach, we highlight the need of referring these patients to centers with experts in melanoma, anorectal and uro-genital cancers treatments. Taking into account the rarity, the poor outcomes and the lack of effective treatment options for MM, tailored research needs to be promptly promoted.
PubMed: 35052829
DOI: 10.3390/biomedicines10010150 -
Anais Brasileiros de Dermatologia 2015Paget's disease, described by Sir James Paget in 1874, is classified as mammary and extramammary. The mammary type is rare and often associated with intraductal cancer... (Review)
Review
Paget's disease, described by Sir James Paget in 1874, is classified as mammary and extramammary. The mammary type is rare and often associated with intraductal cancer (93-100% of cases). It is more prevalent in postmenopausal women and it appears as an eczematoid, erythematous, moist or crusted lesion, with or without fine scaling, infiltration and inversion of the nipple. It must be distinguished from erosive adenomatosis of the nipple, cutaneous extension of breast carcinoma, psoriasis, atopic dermatitis, contact dermatitis, chronic eczema, lactiferous ducts ectasia, Bowen's disease, basal cell carcinoma, melanoma and intraductal papilloma. Diagnosis is histological and prognosis and treatment depend on the type of underlying breast cancer. Extramammary Paget's disease is considered an adenocarcinoma originating from the skin or skin appendages in areas with apocrine glands. The primary location is the vulvar area, followed by the perianal region, scrotum, penis and axillae. It starts as an erythematous plaque of indolent growth, with well-defined edges, fine scaling, excoriations, exulcerations and lichenification. In most cases it is not associated with cancer, although there are publications linking it to tumors of the vulva, vagina, cervix and corpus uteri, bladder, ovary, gallbladder, liver, breast, colon and rectum. Differential diagnoses are candidiasis, psoriasis and chronic lichen simplex. Histopathology confirms the diagnosis. Before treatment begins, associated malignancies should be investigated. Surgical excision and micrographic surgery are the best treatment options, although recurrences are frequent.
Topics: Breast Neoplasms; Carcinoma; Diagnosis, Differential; Female; Genital Neoplasms, Male; Humans; Male; Nipples; Paget Disease, Extramammary; Paget's Disease, Mammary; Skin Neoplasms; Vulvar Neoplasms
PubMed: 25830993
DOI: 10.1590/abd1806-4841.20153189 -
Melanoma Research Aug 2023Malignant vulvar melanoma (VuM) and vaginal melanoma (VaM) represent a unique subgroup of rare malignant melanomas with critical biological properties that differ from... (Observational Study)
Observational Study
Malignant vulvar melanoma (VuM) and vaginal melanoma (VaM) represent a unique subgroup of rare malignant melanomas with critical biological properties that differ from other cancers. In Japan, adequate surveys have yet to be conducted. This study aimed to elucidate the clinicopathological demographics and outcomes of VuM and VaM in Japan. This retrospective observational study included women with invasive VuM or VaM identified from older medical records in Japan. We collected clinical data and used the Kaplan-Meier method to analyze progression-free survival (PFS) and overall survival (OS). Univariate and multivariate regression models were used to identify factors significantly related to survival. We identified 217 patients, 109 (50.2%) with VuM and 108 (49.8%) with VaM. The median PFS was 16.8 months in patients with VuM [95% confidence interval (CI), 23.1-87.7] and 15.6 months in those with VaM (95% CI, 8.4-12.6). The median OS was 43.9 months (95% CI, 60-138) and 31.1 months (95% CI, 24.8-45.3) in patients with VuM and VaM, respectively. Multivariate analysis showed that a disease stage higher than stage III, based on the American Joint Committee on Cancer (AJCC) guidelines, was associated with poorer PFS [hazard ratio (HR), 2.063; 95% CI, 0.995-4.278] and an unknown surgical margin was the only independent factor influencing OS (HR, 2.188; 95% CI, 1.203-3.977). The overall outcomes of invasive VuM and VaM in Japan remain poor. AJCC staging and surgical margins were significant predictors of survival.
Topics: Humans; Female; Melanoma; Skin Neoplasms; Japan; Vaginal Neoplasms; Vulvar Neoplasms; Vagina; Vulva; Demography; Retrospective Studies; Neoplasm Staging; Prognosis; Melanoma, Cutaneous Malignant
PubMed: 37162526
DOI: 10.1097/CMR.0000000000000894 -
Dermatology Practical & Conceptual Apr 2018Vulvar 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....
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.
PubMed: 29785335
DOI: 10.5826/dpc.0802a16 -
Asian Journal of Urology Oct 2022Genitourinary (GU) melanoma is a rare presentation of melanoma accounting for approximately 0.5% of all melanomas. GU melanomas include primary melanomas of the vulva,... (Review)
Review
Genitourinary (GU) melanoma is a rare presentation of melanoma accounting for approximately 0.5% of all melanomas. GU melanomas include primary melanomas of the vulva, vagina, uterine cervix, ovary, penis, scrotum, urethra, bladder, ureter, and kidney. These melanomas are often diagnosed in advanced stages and stigma is thought to contribute to delays in presentation. As the likely diagnosing provider, it is imperative that dermatologists, urologists, and gynecologists are aware of these uncommon sites of presentation. While there have been major advances in the treatment of melanomas as a whole in the last 10 years, their applications to GU melanomas have often been overlooked. GU melanomas have not been included in many of the major phase III clinical trials which brought contemporary advanced treatments to market and the prognoses for GU melanomas remain poor. Due to the rarity of GU melanomas, much of the literature provides generalized recommendations across multiple different organs affected by GU melanomas or omits certain topics, making it difficult to appreciate the fundamentals of the individual presentations. This review aimed to provide background information on the pathogenesis and epidemiology of the different sites of GU melanomas and categorize data specific to the presentation, staging, treatment, and prognosis of each type of GU melanoma to guide the clinician. It was also meant to encourage a multidisciplinary approach to the management of these patients as it spans the expertise of surgical oncologists, medical oncologists, radiation oncologist, dermatologists, urologists, and gynecologists.
PubMed: 36381597
DOI: 10.1016/j.ajur.2022.01.003 -
Journal of Gynecologic Oncology Sep 2020Melanoma comprises 5% to 10% of vulvar cancers and prognosis is poor. The purpose of this study was to identify prognostic factors and treatment patterns for vulvar...
OBJECTIVE
Melanoma comprises 5% to 10% of vulvar cancers and prognosis is poor. The purpose of this study was to identify prognostic factors and treatment patterns for vulvar melanoma using the National Cancer Database (NCDB).
METHODS
The NCDB was queried for patients with invasive vulvar melanoma from 2004-2015. Descriptive statistics were generated to describe clinical and treatment details. Multivariable Cox regression and the Kaplan-Meier method were used to examine overall survival (OS).
RESULTS
1,917 patients with vulvar melanoma met inclusion criteria. Median follow-up time was 32 months (range, 0-151 months). Older age, larger tumor size, advanced disease stage, increased Charlson-Deyo comorbidity score, and care at a non-academic center were independent predictors for decreased OS. Surgical management of the primary site, lymph node surgery, and insurance provided a significant survival benefit. Use of immunotherapy for vulvar melanoma has increased over time. Two-year OS with immunotherapy in patients with distant metastatic disease was higher, although this did not reach statistical significance (33% vs. 12%, p=0.054).
CONCLUSIONS
Vulvar melanoma has a poor prognosis for those with regional and distant metastatic disease. Extent of disease, tumor size, and patient age are important prognostic factors. Other favorable factors included insurance and surgical management. The use of immunotherapy has increased over time and may improve survival in those with distant disease. These data support further investigation into the role of immunotherapy for vulvar melanoma to optimize outcomes.
Topics: Aged; Female; Humans; Medicare; Melanoma; Neoplasm Staging; Prognosis; Retrospective Studies; United States; Vulvar Neoplasms
PubMed: 32808496
DOI: 10.3802/jgo.2020.31.e66 -
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 -
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 -
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