Objective:

To report a case of advanced clear cell adenocarcinoma of the urethra (CCAU) with lymph node metastases that achieved long-term disease control through multimodal therapy, including chemotherapy and surgical intervention.

Approach:

Key Findings:

• Neoadjuvant chemotherapy resulted in a partial response with a 37.5% reduction in lymph node size.
• Postoperative examination confirmed ypT4N2M0 status with negative surgical margins.
• Adjuvant nivolumab was discontinued due to immune-related hypothyroidism and nodal recurrence.
• Resumption of gemcitabine plus cisplatin chemotherapy led to a further 42% reduction in lymph node size.

Interpretation:

Multimodal treatment combining radical surgery and platinum-based chemotherapy may contribute to disease control in advanced CCAU with lymph node metastasis.

Limitations:

• Genomic profiling did not directly guide therapy in this case, and the rarity of CCAU limits the generalizability of findings.

Conclusion:

This case highlights the potential for multimodal treatment strategies in managing advanced CCAU.

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