Clear cell adenocarcinoma of the urethra is a very rare entity. The presented case in a 35-year-old wo-an is the fourth in the literature. The tumour was localized in the wall of the urethra and infiltrated into the outer muscular layer of the urinary bladder. Metastatic spread was found in the paraaortic lymph nodes up to the level of the renal arteries. The final outcome was fatal in spite of the combined treatment with extensive surgery, cytostatic drugs and radiotherapy.
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