Objective: Since the principles of treatment of primary esophageal small cell carcinoma (PESCC) remain still in controversy, the aim of this study was to investigate the clinical characteristics, treatment modalities and prognostic factors of this malignancy.
Methods: The clinical data of 109 patients treated by surgery in our hospital between October 1989 and April 2009 were retrospectively reviewed and analyzed. According to the most recently published TNM staging system for esophageal cancer (AJCC 2009), there were 17 patients in stage Ib, 31 patients in stage II, 59 patients in stage III, and 2 patients in stage IV. All the data were analyzed using SPSS 15.0 software. The median survival time (MST) and overall survival rate (OS) were calculated and compared by the Kaplan-Meier method and log-rank test. The prognostic factors were calculated by Cox hazard regression model.
Results: Among all the 109 patients included, 93 patients were treated by radical esophagectomy, and 11 patients by palliative resection, while 5 patients by exploration. The median survival time (MST) of the whole group was 14.4 months and the 1-, 3- and 5-year overall survival rates (OS) were 56.9%, 17.6%, and 12.0%, respectively. The median survival time (MST) and 5-year overall survival rates (OS) were 18.5 months and 21.4% for pathological N0 cases, 23.5 months and 24.0% for N1 cases, 8.5 months and 0% for N2 cases, and 10.5 months and 0% for N3 cases, respectively (P < 0.001). The MST and 1-, 3- and 5-year OS of patients treated with postoperative chemotherapy were 17.0 months, 60.7%, 19.8%, and 13.0%, respectively, statistically significantly longer than the 7.0 months, 28.5%, 8.9% and 8.9%, respectively, of the patients without chemotherapy (P = 0.005). The pathological N stage and postoperative chemotherapy were independent prognostic factors by Cox multivariate analysis.
Conclusions: Primary esophageal small cell carcinoma is an aggressive systemic disease, characterized by early and wide dissemination of lymph nodes and poor prognosis while treated with surgery or chemotherapy alone. Multimodality treatment based on radical esophagectomy should be recommended for patients in pathological stage I and II.