Abstract
BACKGROUND: Clear cell adenocarcinoma of the urethra is a rare type of aggressive cancer with a poor prognosis. Clear cell carcinoma of the urethra represents less than 0.02% of all malignancies in women. Adenocarcinomas account for 10% of female urethral carcinomas, of which 40% are the clear cell variant. Determining the presence or absence of certain mutations through genetic testing may predict whether a patient with cancer may benefit from a particular chemotherapy regimen. CASE SUMMARY: A 40-year-old woman presented with a 3-year history of slow urinary flow and a 3-mo history of urinary urgency and frequency as well as gross hematuria. An abdominal and pelvic computed tomography scan demonstrated enlarged lymph nodes in the abdomen and pelvis. A biopsy of a left inguinal lymph node microscopically confirmed a metastatic adenocarcinoma of the urethra. Specialized genetic testing determined personalized chemotherapy. She was treated successfully with a non-platinum-based chemotherapy consisting of paclitaxel and bevacizumab. Following 3 cycles of paclitaxel and bevacizumab, she attained significant clinical improvement, and response by FDG-Positron emission tomography (PET) imaging showed a definite improvement in size and metabolic activity. She achieved complete response after 6 cycles of therapy by PET scan. The patient concluded 11 cycles of paclitaxel and bevacizumab, and a subsequent PET scan confirmed progression of metastatic disease. The patient was then treated with two cycles of doxorubicin after which a PET scan revealed a mixed response to the treatment. CONCLUSION: We report the first case of a patient with metastatic clear cell adenocarcinoma of the urethra who underwent personalized chemotherapy after testing for cancer gene alterations. Our unique case represents the safe and effective use of non-platinum-based chemotherapy in clear cell adenocarcinoma of the urethra.