Female urethral carcinoma is among the rarest types of neoplasia of the genitourinary tract and corresponds to 0.003% of all malignant neoplasias occurring in the female urogenital tract. Of the urethral cancers, 70% are squamous cell, 20% are transitional cell, and 10% are adenocarcinoma. Primary urethral adenocarcinoma in women is a rare malignancy of unclear origin.

We present the case of a 69-year-old woman diagnosed in 2015 with an adenocarcinoma of the urethra (pT4N0R1L0Pn1), treated by cystectomy and hysterectomy, adnexectomy and lymphadenectomy followed by radiotherapy. One year later, a PET-CT scan showed micro-nodules in the lungs and a biopsy confirmed the diagnosis of disseminated adenocarcinoma from the urethra. The patient was discussed by our multidisciplinary oncology treatment team and we decided to treat the patient with carboplatin and taxol. A CT scan after three courses of treatment revealed a partial response. It is not clear whether the patient would have benefited from adjuvant chemotherapy since the tumour extent was large.

This is an extremely rare case with no clear guidelines regarding treatment. Options for treatment of female urethral carcinoma include surgery, radiation therapy and chemotherapy, alone or in combination. Treatment has tended toward a multimodality approach in recent years. For advanced female urethral cancer, a combination of chemotherapy, radiation therapy and surgery is recommended for optimal local and distant disease control.