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Female urethra adenocarcinoma (FUA) is a rare aggressive tumor that occurs in Skene ducts and glands. It is associated with a relatively poor prognosis. The aim of this review was to evaluate FUA in terms of epidemiology, risk factors, diagnosis, natural history of disease, modalities of treatment, and outcomes. These tumors are usually large masses, which typically spread through the lymphatic system. Patients present with vague symptoms similar to urinary tract infections. Cystourethroscopy permits visualization of the urethral tumor and allows biopsies to be performed to remove samples for histologic examination. Magnetic resonance imaging is recommended for tumor staging. Local, superficial, and distal urethral tumors may be treated by partial resection with preservation of the urethra. Radical urethrectomy with wide, comprehensive resection of the paraurethral tissues and anterior vaginal wall may offer superior local control for this disease. Advanced FUA and lymph node positivity are associated with poor prognosis for all survival outcomes (recurrence-free, cancer-specific, and overall survival). Multimodal therapy including surgery, chemotherapy, and radiotherapy is required in the modern management of FUA, although the specific role and combination of each treatment is less clearly determined. Wide resection after chemotherapy and/or radiotherapy is associated with the best local control, but it reduces quality of life.