%0 Journal Article %T The epidemiology and survival of extrapulmonary small cell carcinoma in South East England, 1970¨C2004 %A Yien Wong %A Ruth H Jack %A Vivian Mak %A M£żller Henrik %A Elizabeth A Davies %J BMC Cancer %D 2009 %I BioMed Central %R 10.1186/1471-2407-9-209 %X We used data from the Thames Cancer Registry database for South East England between 1970 and 2004 to determine the incidence, most common anatomical sites, and survival by site, and stage of EPSCC. 1618 patients registered with EPSCC were identified. We calculated the age-standardised incidence rate for EPSCC using the European standard population and compared this to that for SCLC. We calculated survival using the Kaplan-Meier method for EPSCC and SCLC, and reported 3-year survival for different EPSCC anatomical sites and disease stages.The incidence of EPSCC was much lower than for SCLC, similar in males and females, and stable throughout the study period, with incidence rates of 0.45 per 100,000 in males and 0.37 in females during 2000¨C2004. In general, patients with EPSCC had a better 3-year survival (19%) than SCLC (5%). The most common anatomical sites for EPSCC were oesophagus (18%), other gastrointestinal (15%), genitourinary (20%), head and neck (11%), and breast (10%). Breast EPSCC had the best 3-year survival (60%) and gastrointestinal EPSCC the worst (7%).This study suggests that EPSCC has a stable incidence and confirms that it presents widely, but most commonly in the oesophagus and breast. Site and extent of disease influence survival, with breast EPSCC having the best prognosis. Further studies using standardised diagnosis, prospective case registers for uncommon diseases and European cancer registries are needed to understand this disease.Neuroendocrine tumours can be broadly classified into three groups: well differentiated tumours (true carcinoids), moderately differentiated tumours (atypical carcinoids) and poorly differentiated tumours (small cell carcinomas) [1]. The latter group includes extrapulmonary small cell carcinoma (EPSCC) and small cell lung cancer (SCLC). EPSCC is a rare entity, and in the United States it accounts for approximately 2.5 to 5% of all small cell carcinomas [2-4]. The term came into use in the 1990s and various descrip %U http://www.biomedcentral.com/1471-2407/9/209