ObjectiveTo investigate the clinical characteristics,diagnosis,treatment and prognosis of limited stage primary esophageal small cell carcinoma (PESC).MethodsClinical data was retrospectively analyzed for 42 patients with pathologically confirmed PESCs who underwent transthoracic esophagectomy with lymphadenectomy from Nov.1990 to Dec.2010 at the Cancer Hospital of Shantou University Medical College.The survival analysis was performed using Kaplan-Meier method.ResultsThe clinical symptoms,imaging and endoscopic features of PESC were similar to those of esophageal squamous cell carcinoma (ESCC).Of the 26 cases that received pre-operative endoscopic biopsy,only five cases were diagnosed as PESC,while the other 21 cases were misdiagnosed as ESCC.The mean follow-up time of this series was 25.3 months (0-123 months).34 patients died of the disease during the follow-up;7 were still alive and 1 was lost.The median survival time (MST) of the 41 patients was 13.0 months (95 ％ confidence interval 6.3-19.7),and the 6-,12-,24-,36-,and 60-month overall survival rates (OS) were 78.6 ％,57.5 ％,30.8 ％,23.7 ％,10.5 ％,respectively.ConclusionPESC is a rare disease with poor prognosis,and is prone to be misdiagnosed by endoscopic biopsy.Currently no standard treatment has been established.