Clinical features of leiomyosarcoma of the urinary bladder: Analysis of 183 cases

Abstract

IntroductionExperience with management of urinary bladder leiomyosarcoma (LMS) is rare. Therefore, to better elucidate the disease characteristics of urinary bladder LMS, we utilized a large population-based cancer registry to examine the epidemiology, natural history, pathological characteristics, prognostic factors, and treatment outcomes. Material and methodsThe Surveillance, Epidemiology, and End Results database (1973–2010) was used to identify cases by tumor site and histology codes. The association between clinical and demographic characteristics and long-term survival was examined. ResultsA total of 183 histologically confirmed cases were identified between 1973 and 2010. The annual age-adjusted incidence rate was 0.23 cases per 1,000,000 and did not significantly change over time. Median age of the patients was 65 years (interquartile range: 47–78y). Of the patients with a known pathologic tumor stage (n = 164), 50% had a regional or distant disease. Overall, 63.2% of patients with known histologic grade (n = 106), had poorly differentiated or undifferentiated histology. Most patients (92.9%) received cancer-directed surgery (CDS), with 34.4% having radical or partial cystectomy. Only 7.7% of patients received radiation therapy in combination with surgery. The median disease-specific survival was 46 months. Five- and 10-year cancer-specific survival rates were 47%, and 35%, respectively. On multivariate analysis, a worse outcome was associated with an undifferentiated tumor grade, distant disease, and failure to undergo CDS. ConclusionThis series represents the largest cohort of LMS of the urinary bladder studied to date. LMS commonly presented as high grade and advanced stage with a poor prognosis. Reduced disease-specific survival was associated with increasing age, undifferentiated tumor grade, distant disease, and failure to undergo CDS.