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

Authors Zhong W, Lin R, Zhang L, Jin C, Li X, He Q, Gong K, He Z, Zhou L

Received 4 April 2017

Accepted for publication 6 July 2017

Published 17 August 2017 Volume 2017:10 Pages 4105—4111

DOI https://doi.org/10.2147/OTT.S138769

Checked for plagiarism Yes

Review by Single-blind

Peer reviewers approved by Dr Colin Mak

Peer reviewer comments 2

Editor who approved publication: Dr Jianmin Xu

Wenlong Zhong,* Rongcheng Lin,* Lei Zhang, Chengyue Jin, Xuesong Li, Qun He, Kan Gong, Zhisong He, Liqun Zhou

Department of Urology, Peking University First Hospital, Institute of Urology, Peking University, National Urological Cancer Center, Beijing, People’s Republic of China

*These authors contributed equally to this work

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.

Keywords: carcinoma, neuroendocrine, small cell, ureteral carcinoma, systematic review
 

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