Abstract
BACKGROUND: To review the experience of diagnosis and treatment of primary small cell carcinoma (SCC) in our institution and discuss the clinicopathologic characteristics, treatments and outcomes of patients with primary ureteral SCC. METHODS: Patients diagnosed with ureteral SCC in our institution from January, 2007 to December, 2016 were reviewed. In addition, we performed a systematic literature review in October 2016 on case reports and case series of ureteral SCC. The clinicopathologic characteristics, treatments and outcomes of this rare disease were analyzed. RESULTS: A total of 32 patients were included in our analysis (4 cases from our institution and 28 cases from the literature). Most patients (71.0%) were male with an average age of 66.6 years (range, 48–80 years). The most common symptoms were hematuria (n=14, 48.3%) and flank pain (n=14, 48.3%). All patients received surgery, with 12 (37.5%) patients underwent multimodality therapy. Regional or distant recurrence occurred in 11 patients, among which only 1 patient presented bladder recurrence. The overall median survival for the patients was 17 months, with a 1- and 3-year survival rates 51.9% and 30.3%, respectively. In a univariate analysis, female (P=0.009), pure SCC (P=0.03), advanced T stage (P=0.04) were associated with worse overall survival. CONCLUSIONS: Ureteral SCCs are extremely rare neoplasms with aggressive natural history and poor prognosis. T stage, tumor components and gender may be important factors influencing prognosis. A multimodality treatment is recommended for the management. However, further studies are needed to improve the treatment strategy.