Methods: Of a total of 953 patients diagnosed with esophageal cancer between January 2000 and M arch 2014 in Atatürk University, Faculty of Medicine, Department of Thoracic Surgery, 37 patients (14 males, 23 females; mean age 63.7±12.1 years; range 20 to 87 years) with rare primary malignant tumors of the esophagus were included in the study. We assessed the patients retrospectively according to age, sex, histological type, tumor localization, treatment administered, tumor stage, and survival. Esophagoscopy, tomography, esophageal barium X-ray, abdominal ultrasonography, and positron emission tomography and computed tomography were performed for diagnosis and staging purposes.

Results: Of the patients, 19 had adenosquamous cell carcinoma, nine had neuroendocrine cell carcinoma, four had basaloid squamous cell carcinoma, two had carcinosarcoma, one had mucoepidermoid carcinoma, one had adenoid cystic carcinoma, and one had leiomyosarcoma. Due to limited disease, esophagectomy was performed to 19 patients. Survival rate was better in patients who were performed esophagectomy (p<0.05).

Conclusion: In terms of clinical features, rare tumors of the esophagus have common features with squamous cell carcinoma and adenocarcinoma of the esophagus. In these tumors, the most important factor affecting survival is the esophagectomy performed at an early stage.