Abstract
BackgroundExtrapulmonary small cell carcinoma (EPSCC) is a rare cancer and few studies describe its epidemiology. Our objectives were to compare the incidence and survival of EPSCC in South East England with small cell carcinoma of the lung (SCLC), to determine the most common anatomical presenting sites for EPSCC and to compare survival in EPSCC by disease stage and site of diagnosis.MethodsWe 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.ResultsThe 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 2000–2004. 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%).ConclusionThis 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.
Highlights
Extrapulmonary small cell carcinoma (EPSCC) is a rare cancer and few studies describe its epidemiology
The incidence of small cell carcinoma of the lung (SCLC) remained higher in males than in females it declined in males and increased in females during the study period
This study of 1618 patients registered with EPSCC in South East England is one of the largest studies so far published on this clinicopathological entity
Summary
Extrapulmonary small cell carcinoma (EPSCC) is a rare cancer and few studies describe its epidemiology. 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]. There is recent molecular evidence that small cell neoplasms may occur as a late-stage phenomenon in genetically associated organ-typical carcinomas [9]. Despite these new findings, EPSCC is still poorly appreciated and clinically it may be confused with metastases from SCLC
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