Abstract
BACKGROUND: Urethral malignant melanoma is an uncommon and aggressive malignancy, frequently diagnosed at an advanced stage, posing diagnostic and therapeutic challenges. CASE PRESENTATION: We present the case of a 74-year-old North African female diagnosed with primary metastatic urethral melanoma. Initial staging included a thorough physical examination, pelvic MRI, and whole-body CT scan, initially showing no signs of distant metastasis. Subsequent FDG PET/CT revealed unexpected bone metastases, prompting a significant shift in our management approach. The patient received immunotherapy with pembrolizumab, resulting in sustained metabolic stability at the six-month follow-up. DISCUSSION: We present recent epidemiological data and risk factors for this uncommon melanoma site. Additionally, we delve into the diagnostic challenges, underlining the expanding role of PET/CT. The discussion also covers managing options in the absence of a consensus. CONCLUSION: Despite several treatment choices, metastatic urethral melanoma still has a poor prognosis. This reality reflects the complexity of the disease and emphasizes the need for further research to unravel its underlying mechanisms and to establish more effective therapeutic approaches.