Abstract
INTRODUCTION: Esophageal sarcoma is a rare neoplasm of the gastrointestinal tract. The majority of the esophageal neoplasms have an epithelial origin. In this report, we present a case of a middle-aged man with an enormous spindle cell sarcoma whose symptoms initiated only a few weeks before diagnosis. PRESENTATION OF CASE: A 41-year-old man with an unremarkable past medical history and physical examination presented with recent aggravation of cough and severe, progressive dysphagia to solid foods resulting in a 25-kilogram weight loss without any prior symptoms. He had no history of cigarette smoking and alcohol consumption. The CT scan showed a huge soft tissue mass with heterogeneous enhancement from the proximal esophagus to 4 cm above the gastro-esophageal junction, causing luminal bulging. Trans-hiatal esophagectomy and gastric pull-up were performed. Pathology report confirmed the diagnosis of sarcoma. Further pathological evaluation using immune-histochemical studies, confirmed the tumor as spindle cell sarcoma. The postoperative period was uneventful, and there were no signs and symptoms related to tumor recurrence one year after surgery. DISCUSSION: The most challenging aspect of diagnosing sarcomas is differentiating them from other pathologies, such as gastrointestinal stromal tumors, synovial sarcomas, sarcomatoid carcinomas, melanomas, and solitary fibrous tumors. Immunohistochemical studies play a vital role in this differentiation. Additionally, cytokeratin AE1/AE3 has been introduced as a marker of epithelial differentiation and can verify the presence of the epithelial component in tumors, such as in carcinosarcomas. CONCLUSION: Considering the potential for an unusual size, sarcoma should be considered in a differential diagnosis for huge esophageal masses.