Gastrointestinal stromal tumors (GISTs) are the most common sarcoma of the gastrointestinal tract, but occur rarely in the esophagus. Use of tyrosine kinase inhibitors has revolutionized therapy for GISTs but complete resection remains the treatment of choice. Esophageal GISTs require special consideration regarding perioperative treatment, evaluation, and conduct of operation.

We report our experience (from 2000 to 2003) with four patients who underwent resection of esophageal GIST, reviewed consensus management guidelines, and performed a literature review for reported cases. Query of the National Cancer Database was made to assess national experience with esophageal GISTs.

All four patients had esophageal GISTs successfully differentiated from leiomyomata by immunohistochemistry on fine needle aspirates obtained during endoscopic ultrasound. At the time of resection all tumors were noted to be adherent to tissues other than submucosa that required resection of esophageal muscularis, periesophageal tissue, or mucosa. All patients were thought to have complete resection and were alive at last contact (median, 33 months). Two of four patients recurred, one of whom underwent esophagectomy; the other patient is alive on imatinib. Seven case reports with median follow-up of three years reported no recurrence with either enucleation or esophagectomy. The National Cancer Database contains 33 cases of esophageal GIST reported since 1999. Nine of 24 patients died within six months of diagnosis.

Esophagectomy is the treatment of choice for esophageal GISTs but requires attention to operative details that emphasize complete en bloc excision of surrounding tissue. Preoperative imatinib may improve resectability.