A rare entity of esophageal gastrointestinal stromal tumor with literature review.

Gastro-esophageal Stromal Tumor (GIST) is the type of mesenchymal tumor that arises from specialized cells called interstitial cells of Cajal (ICC) or their precursors. The most common site is stomach (60%-70%) followed by small intestine (20%-30%). Esophageal GISTs comprises 0.7% of all GISTs, making them incredibly rare. Treatment options for esophageal GISTs typically involve a multidisciplinary approach and depend on the several factors, including the tumor size, location, and the presence of metastasis. The primary treatment for localized esophageal GISTs is surgical removal, often involving a partial or complete esophagectomy. In some cases, targeted therapy with drugs such as imatinib may be used before or after surgery to shrink the tumor or prevent its recurrence. We report a case of middle-aged man, who presented with dysphagia and was diagnosed as gastrointestinal stromal tumor of lower thoracic esophagus, positive for c-kit, and SMA, and negative for desmin. Patient was treated with targeted therapy and surgery that resulted in excellent outcome with prolonged survival. Although esophageal GIST is a rare scenario, outcome in this subset of patients can be improved with the use of targeted therapy that hasten the symptomatic relief and adds to survival benefit.