Primary urethral cancer is recognized to be a rare malignant tumor which comprises less than 1% of all tumors. In 2008 there were 4292 cases in European Union with potential incidence of 655 new cases per year. A prevalent histological type of primary urethral cancer is the urothelial carcinoma (54-65%), followed by squamous cell carcinoma (16-22%) and adenocarcinoma (10- 16%).

In Europe median of five-year OS has no difference between male and female patients. According to RARECARE (rare tumors observation project), median 1-year and 5-year OS of patients with urethral cancer was 71% and 54% respectively. A more lengthy observation in SEER (National Cancer Institute program – a source of information on epidemiology, prevalence and survival for cancers in USA), which included 1615 cases of primary urethral cancer, reported median 5-year and 10-year OS being 46% and 29% respectively. Cancer-specific survival for 5 and 10 years was 68% and 60% respectively.

Multimodal approach to treatment for primary urethral cancer includes radical surgery and chemotherapy with possible adjuvant radiation therapy, which allows to achieve satisfactory long-term survival. Considering a possibility of multiple histological types of urethral cancer in one patient an adequate pathology using all techniques for morphological diagnostics is necessary for guiding treatment algorithm.

Authors declare lack of the possible conflicts of interests.