Aim: Primary esophageal small cell carcinoma is a comparatively rare disease. It is thought to have a poor prognosis, and no standard treatment strategy has yet been established. Surgical cases of small cell carcinoma of the thoracic esophagus were reviewed with regard to clinical characteristics and treatment strategy. Patients and methods: Among the 993 patients who underwent an esophagectomy for thoracic esophageal cancer from 1965 to 2007, 12 cases (1.2%) demonstrated a diagnosis of small cell carcinoma based on the histopathology findings. Results: There were 3 cases of Stage I, 1 case of Stage II, 4 cases of Stage III, and 4 cases of Stage IVa. Five cases (42%) were treated preoperatively (chemoradiotherapy, 4 cases; radiotherapy, 1 case), and the pathological criteria for the effects of preoperative therapy were 3 cases of Grade 3. Eleven patients died of cancer; only 1 patient achieved long-term survival and complete response for treatment with chemoradiation after recurrence. The median survival time was 7.6 months, the 1-year survival rate was 33%, and the 3-year survival rate was 8.3%. Conclusions: The prognosis of surgically resected cases of esophageal small cell carcinoma is poor; therefore, either chemotherapy or chemoradiotherapy should be administered to such cases. Furthermore, improved understanding of the biological characteristics of this type of tumor will result in the development of new therapeutic strategies, which are expected to improve patient outcome.
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