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A rare bladder cancer - small cell carcinoma: review and update

DOI: 10.1186/1750-1172-6-75

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Abstract:

Small cell carcinoma of the bladderPoorly differentiated neuroendocrine carcinoma of the bladderSmall cell carcinoma of the bladder (SCCB) is a rare, poorly differentiated neuroendocrine epithelial tumour associated with a more aggressive behaviour and poorer outcome than bladder transitional cell carcinoma (TCC). It is mostly diagnosed at advanced stage and generally believed to have a high metastatic potential. Current knowledge of this disease is limited and was based mainly on retrospective investigations. The disease was initially described in 1981 by Cramer et al [1]. Bladder small cell carcinoma (SCC) is frequently found combined with other histological forms of bladder cancer: TCC, adenocarcinoma and squamous cell carcinoma [2-10]. The pathogenesis of primary SCCB is unknown. However, several hypotheses were proposed to explain the origin of SCC in the bladder. The most important hypothesis was: the origin of SCCB may be a multipotential common stem cell. Treatment of SCCB is extrapolated from the treatment of small cell lung carcinoma (SCLC). This comprehensive review would provide a real insight into the epidemiology, pathogenesis, diagnosis, staging, treatment, and prognosis of SCCB.We based our review on the MEDLINE database using the key words 'bladder cancer', 'small cell carcinoma', 'pathogenesis', 'diagnosis', 'treatment', and 'prognosis'. The research was performed since January 1980 up to July 2011. Only one prospective phase II study was reported in the English literature. Twenty retrospectives studies including ≥ 20 patients have been reported. There have also been several interesting case reports and literature reviews.Small cell cancer of the bladder is an extremely rare bladder malignancy with a mean frequency of 0.7% and a range between 0.35% and 1.8% [2-7]. The reported incidence is less than 1-9/1,000,000 habitant. Since 1980, less than 1000 cases of SCCB have been diagnosed and reported in the literature up to July 2011. The demographic ch

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