Malignant melanoma of the vulva is a rare malignant disease. Women in their 50s, 60s, and 70s are predominantly affected. The clinical presentation can be in the form of an asymptomatic pigmented lesion, or with manifestations of vaginal bleeding, discharge, local pain, itching, and urinary discomfort. The disease has the aggressive nature and poor prognosis. A detailed physical examination followed by a histopathological evaluation of the lesion is crucial for making the diagnosis.
Malignant melanoma of the vulva often has an insidious course and carries a poor prognosis because of its high potential for spreading to distant tissues   . One of its peculiar characteristics is the absence of ultraviolet (UV) light exposure as a risk factor  . This rare tumor (< 5% of all melanomas in women) most often presents as an asymptomatic pigmented lesion, with the principal locations being the labia majora and the clitoral region, but the labia minora, the periurethral area, and, very rarely, the introitus of the vagina are also possible locations   . The glabrous skin is much more frequently the site of malignant melanoma of the vulva compared to hairy skin . When manifestations are present, vaginal bleeding is reported as the most common, whereas vaginal discharge, pruritus, pain, vaginal irritation, and a stinging sensation are other notable symptoms and signs    . Weight loss may be reported in some cases . Malignant melanoma of the vulva is mainly identified in elderly women in their sixth, seventh, and eighth decades of life   .
A detailed patient history and a full physical examination are crucial steps of the workup for patients in whom malignant melanoma of the vulva is suspected. Signs and symptoms are often missed during the initial workup , which may significantly affect the prognosis and the efficacy of therapy. The physician must ask about the presence of vaginal-related symptoms, such as bleeding, discharge, or pruritus, whereas a detailed genital examination is necessary to identify a pigmented lesion. The exact type of tumor must be identified as soon as the lesion is detected and a biopsy with subsequent histopathological examination is vital in this regard    . Typical mutations include C-KIT and NRAS genes, whereas BRAF gene mutations are rarely seen . When a suspicion toward the existence of metastases exists (the urethra, vagina, perianal area, regional and distant lymph nodes, liver, and lungs are frequently involved locations), imaging studies need to be conducted in order to determine the extent of tumor spread  . Positron emission tomography-computed tomography (PET-CT) is regarded as the optimal study for evaluating metastatic malignant melanoma of the vulva .