Primary lymphoepithelioma like carcinoma (LELC) of the esophagus is a very rare disease; only 29 cases were reported in the medical literature. This type of tumor is characterized by undifferentiated cells mixed with dense lymphocytic infiltration. Here we would like to present a 52 year old male patient who was diagnosed with stage IV (cT3N2M1) LELC of the middle to lower esophagus. The patient's chief complaint was dysphagia of 3 weeks duration. The patient was successfully treated with concurrent chemotherapy (PF4 regimen) and radiotherapy (5040 cGy). A repeat esophageal biopsy done 12 months after concurrent chemoradiation therapy (CCRT) revealed no evidence of tumor. This patient is alive and disease-free 24 months after CCRT.
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