Abstract
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.
Highlights
Melanoma is an extremely aggressive tumor with a high metastatic rate, whose diagnosis in advanced stages was associated, until a decade ago, with minimal chances of survival [1]
Regarding the distribution according to sex, in the case of melanomas discovered in adulthood, there is a predominance of cases in males, with a reversal of the phenomenon for melanomas diagnosed between 15–39 years, over 60% of cases appearing in the female population [6,7]
Unlike cutaneous melanoma, which is much more common in Caucasians, the frequency of vulvar and vaginal melanomas has very little variability related to race, but there is a slight increase in frequency in Whites (3.14 vs. 1.02: 1) [22]
Summary
Melanoma is an extremely aggressive tumor with a high metastatic rate, whose diagnosis in advanced stages was associated, until a decade ago, with minimal chances of survival [1] It is a tumor originating in melanocyte cells which are formed during embryogenesis from the neural crest of the trunk [2]. Unlike cutaneous melanoma, which is much more common in Caucasians, the frequency of vulvar and vaginal melanomas has very little variability related to race, but there is a slight increase in frequency in Whites (3.14 vs 1.02: 1) [22]. The therapeutic means used in cutaneous melanomas are of little use in the treatment of melanomas with vulvar and vaginal localization, mortality having high values and not changing considerably in the last three decades (5-year survival rates vary between 10% and 63%) [17,24,25,26]. The present review aims to highlight the main risk factors identified in the occurrence of vulvar and vaginal melanomas, clinical manifestations, molecular changes underlying these neoplasms, as well as the main therapeutic means and their effectiveness in terms of survival
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