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

Abstract

PurposeThe 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 methodsPatients 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.ResultsA 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.ConclusionUreteral 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.

Clinical Characteristics, Treatment Strategy, and Outcomes of Primary Large Cell Neuroendocrine Carcinoma of the Bladder: A Case Report and Systematic Review of the Literature

“…In our study, almost half of the patients (43.6%) of LCNEC of the bladder coexisted with other tumor components, such as UC and SCC, which favors the hypothesis of the origin of multipotent stem cells. Not the same as USCC reported by Zhong et al (40), there was no difference in OS between the pure LCNEC and mixed LCNEC groups. We think there are two probable reasons why no difference was found between the two groups.…”

“…Approximately 3/4 of patients with bladder cancer present with non-muscle-invasive bladder cancer (NMIBC), a disease confined to the mucosa (Ta, CIS), or submucosa (T1) (40). However, for LCNEC of the bladder, only 1 (3.3%) patient was classified as NMIBC.…”

Primary small cell carcinoma of the ureter

“…The overall median survival is 17 months, with a 51.9% of 1-year survival rate. [ 3 ] The primary treatment is surgical remotion of the tumor by ureterectomy or nephroureterectomy depending on the clinical status of the patient, stage, and localization of the tumor. Due to the aggressive course of SCC, the surgeon must address the radicality of the excision as early as possible, considering that in most cases the resection alone does not seem to stop the progression of the disease.…”

“…Small cell carcinoma of the ureter has been described in about 40 patients so far [ 3 – 35 ] with similarities in symptoms, management, and outcome, as outlined in Table 1 .…”

Collision Carcinoma Involving Small Cell Neuroendocrine Carcinoma and Squamous Cell Carcinoma of the Ureter: A Case Report and Review of the Literature

“…We report the case of a patient with a ureteral collision tumour consisting of SCNEC and SCC. Here, we systematically review the literature on ureteral collision carcinoma involving SCNEC and summarize the clinicopathological features, current practice of management and prognosis of this rare tumour (2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15).…”