Primary small cell carcinoma of the esophagus (SmCC) is an uncommon aggressive tumor characterized by early systemic dissemination and poor prognosis, regardless of the methods of treatment. The optimal treatment strategy remains uncertain. A retrospective study was conducted to review the results of non-operative treatment for patients with limited and metastatic esophageal SmCC. Between 1993 and 2003, 10 patients were diagnosed to have primary esophageal SmCC in our institution. Six of them had disseminated diseases, whereas the other four had limited disease upon diagnosis. All patients were managed non-operatively by either chemotherapy and/or radiotherapy. The overall median survival was 8 months (range, 2-62 months). The survival was 4-62 months for patients with limited disease, whereas it was 2-10 months for patients with disseminated disease at initial diagnosis. In summary, the current study demonstrated satisfactory palliation could be achieved with chemo-radiation for patients with limited disease; however, the ultimate role of primary chemo-radiation for esophageal SmCC must await results from randomized trials.

Small-cell carcinoma of the esophagus and gastroesophageal junction: review of the Memorial Sloan-Kettering experience.Ann Oncol. 2008 Mar;19(3):533-7. Epub 2007 Oct 17.

BACKGROUND: Esophageal small-cell carcinoma (SCC) is rare, highly malignant and the optimal treatment approach has not been defined. PATIENTS AND METHODS: We report the largest single-institution retrospective review of patients with esophageal and gastroesophageal (GE) junction SCC. RESULTS: Twenty-five patients were identified, with complete records available for 22. Eighty-two percent were male, 82% had pure SCC histology and 86% of tumors were in the lower esophagus or GE junction. On the basis of the Veterans' Administration Lung Study Group criteria, 14 patients (64%) presented with limited disease (LD). Median survival was 19.8 months (range, 1.5 months to 11.2+ years); for LD patients, 22.3 months (range, 6 months to 11.2+ years); for extensive disease (ED) patients, 8.5 months (range, 1.5 months to 2.2 years, P = 0.02). With a median follow-up of 38 months, six patients (27%) are alive, one with ED and five with LD. Two LD patients are alive and free of disease for >5 years. Four of the five LD patients who are long-term survivors received induction chemotherapy followed by chemoradiotherapy without surgery. CONCLUSIONS: Our data indicate that patients with LD esophageal SCC treated with induction chemotherapy followed by consolidative chemoradiation can achieve long-term survival. The contribution of surgery remains unclear.