ABSTRACT
Malignant mucosal melanoma (MM) is a rare and aggressive neoplasm, which behaves differently from its cutaneous counterpart. MM of head and neck account for 55.4%, genital tracts - 18%, ano-rectal - 23.8% and urinary tract - 2.8%. Less than 200 cases of urethral melanomas have been reported in literature, out of which MM of female urethra represent only 0.2% of primary melanomas. Owing to the delayed presentation, early onset of metastasis and aggressive tumor biology, prognosis has remained largely poor despite adequate local control in MM. Surgery is the mainstay of management, with adjuvant radiation contributing to local control but not overall survival benefit. Chemotherapy and immunotherapy options are being explored in both adjuvant and palliative setting. We hereby, report the clinical presentation, management, and course of a rare case of urethral melanoma in a young female patient. After ruling out metastatic disease at presentation, local control was achieved by wide local excision with distal urethrectomy and modified Singapore flap reconstruction along with bilateral modified superficial inguinal block dissection; followed by adjuvant radiotherapy in view of close margins. However, she developed distal recurrence three months post adjuvant therapy, thus reflecting the aggressive biology of this class of rare tumor.
