Abstract

BackgroundMucosal melanomas account for 17% of melanomas, and less than 1% affect the urogenital tract. Primary urethral melanoma is extremely rare; less than 200 cases have been reported so far since it was first described. The clinical presentation is usually delayed, and the prognosis is generally poor.Case presentation.An 86-year-old female was referred to urology due to the presence of a three-month-old violaceous mass in the urethral meatus protruding through the vagina. On physical examination, a pedunculated, hyperpigmented, friable, and tender lesion was seen in the urethral meatus bulging between the labia minora. After initial diagnostic studies, the patient was taken to surgical resection of the lesion. The pathology report identified the lesion as a malignant melanoma of the urethra, and staging studies revealed regional lymph node metastases. After discussing the treatment options with the patient, palliative therapy with nivolumab was started. In follow-up at 26 months, the patient had evidence of extensive lymph node involvement, but a conserved performance status and no visceral metastases.ConclusionPrimary melanoma of the female urethra is an uncommon disease with a poor prognosis. Despite the deficiency in literature regarding its management, it is important to consider patient expectations and preferences when treating this rare disease.

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