Abstract
INTRODUCTION: Gastrointestinal stromal tumor (GIST) is a common malignancy of the gastrointestinal tract, but rarely occurs in the esophagus, composing approximately 0.7% of all GISTs. Surgery is a mainstay treatment for this disease. We herein report a case of a 68-year-old male patient treated by thoraco-laparoscopic esophagectomy followed by imatinib therapy for a large esophageal GIST, and also review a literature regarding this disease. CASE REPORT: A 68-year-old male was admitted to our hospital due to dysphagia. After the initial investigations, the patient was diagnosed with esophageal GIST and underwent thoraco-laparoscopic esophagectomy. Final immunohistochemistry (IHC) was positive with CD117, CD34, DOG-1, Ki67 without the expression of SMA, S-100 that confirmed GIST. The patient was referred to adjuvant therapy with imatinib and there was no recurrence at 16 months after surgery. DISCUSSION: Due to the rarity of esophageal GISTs, diagnosis and treatment remain challenging. Without immunohistochemistry, this disease may be misdiagnosed as esophageal leiomyoma, or with other mesenchymal tumors such as sarcoma or schwannoma due to similar clinical, endoscopic and radiographic characteristics. Surgery is the cornerstone treatment, the choice of enucleation or esophagectomy depends on the comprehensive evaluation of clinical and pathologic factors of the specific case. Neoadjuvant or adjuvant treatment with imatinib is an effective therapy for selective patients. CONCLUSION: GISTs are rare neoplasms of the esophagus. Currently, surgical resection is the cornerstone treatment, adjuvant or neoadjuvant therapy with imatinib may be indicated for selective patients. Clinical trials are expected with longer follow-ups to develop concrete guidelines for this disease.