Clinicopathologic characteristics, therapy and outcomes of patients with primary ureteral small cell carcinoma: a case series and systematic review of the literature.

PURPOSE: The aim of this case series was to review the standard diagnosis and treatment procedures of primary small cell carcinoma (SCC) in our institution and discuss the clinicopathologic characteristics, treatments and outcomes of patients with primary ureteral SCC.

PATIENTS AND METHODS: Patients diagnosed with ureteral SCC in Peking University First Hospital, Beijing, China, from January 2007 to December 2016 were included. 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 underwent surgery, with 12 (37.5%) patients undergoing multimodality therapy. Regional or distant recurrences developed in 11 patients, among which only 1 patient had bladder recurrence. The overall median survival of the patients was 17 months, with 1- and 3-year survival rates of 51.9% and 30.3%, respectively. In a univariate analysis, female (P=0.009), pure SCC (P=0.03) and advanced T stage (P=0.04) were associated with worse overall survival.

CONCLUSION: 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 management. However, further studies are needed to improve the treatment strategy.