Small cell carcinoma of the esophagus: The University of Texas M.D. Anderson Cancer Center experience and literature review

BACKGROUND. Small cell carcinoma of the esophagus is a rare disease with aggressive behavior and poor prognosis. Multidrug chemotherapy remains the treatment of choice given the systemic nature of the disease. Radiotherapy has been used concurrently with chemotherapy to enhance local control. The role of surgery in patients with limited disease is controversial. Limited data exist regarding the pathologic response of the tumor to chemoradiotherapy. The goal of the current study was to analyze the outcome of 8 patients treated at the M.D. Anderson Cancer Center, with particular focus on the histologic findings of the resected specimens. METHODS. Patient records were reviewed for demographics, presenting symptoms, diagnostic modalities, disease stage, treatment, and outcome. RESULTS. Two of eight patients had metastatic disease at the time of diagnosis and received combination chemotherapy. Six patients had limited stage disease. Four received combined modality treatment including esophagectomy, and two received radiotherapy only. All four patients who underwent esophagectomy had pure small cell carcinoma histology at diagnosis and received preoperative combination chemotherapy with or without radiotherapy. None of the four patients achieved a pathologic complete remission. Two patients had residual small cell carcinoma; one patient had squamous cell carcinoma and one adenocarcinoma. The median overall survival for the group of patients was 12.5 months (range, 5-57 months). CONCLUSIONS. In selected patients with limited stage disease, surgery with curative intent should be considered as part of multimodality treatment.