Clinicopathological Profile of Pure Neuroendocrine Neoplasms of the Esophagus: A South Indian Center Experience
BACKGROUND Small cell carcinoma of the esophagus is a rare disease, characterized by aggressive progression. It has a high incidence of metastatic disease at presentation and a poor overall prognosis. Treatment protocols are not well established because of the paucity of cases and a lack of large studies. METHODS We performed a retrospective review of all patients with small cell carcinoma of the esophagus diagnosed at the Tata Memorial Hospital between 1985 and 2001. We retrieved and analyzed data regarding demographic details, diagnosis, staging, type of treatment, and overall survival. RESULTS Eighteen patients with a mean age of 62 years (range 48 to 80 years) diagnosed as having small cell carcinoma of the esophagus were analyzed. The group included 13 men and 5 women. Seven of the 18 patients (39%) presented with metastatic disease including 5 patients (28%) with liver metastases. Four patients were treated with surgery, with or without chemotherapy or radiotherapy. Three patients were treated with combination chemoradiotherapy, 2 patients with chemotherapy alone, and 5 patients with radiotherapy alone. Four patients with advanced disease and poor general condition were not offered any treatment. The overall median survival of our patients was 6 months. Patients treated with surgery and chemotherapy had a better overall survival. CONCLUSIONS Small cell carcinoma of the esophagus should be regarded as a systemic disease with a high distant failure rate. Treatment strategies hence must incorporate systemic chemotherapy along with radical surgery or radiotherapy as part of a multimodality approach.