Background: As esophageal gastrointestinal stromal tumors (GISTs) are very rare, their clinicopathological features remain obscure. We conducted a nationwide survey to clarify the characteristics of these tumors and to establish a consensus on their diagnosis and treatment.
Methods: The clinicopathological information of patients with esophageal GISTs who underwent treatment between January 2010 and June 2016 at the accredited institutions by the Japan Esophageal Society was collected via a questionnaire method and analyzed statistically.
Results: Fifty-one patients (29 men and 22 women; median age, 68 years) were recruited from 31 institutions. Diagnosis was triggered most frequently during screening and other disease scrutiny. Symptoms were seen only in 17 patients: highest in 11 patients with dysphagia. Thirty-five patients underwent surgery alone; 15 patients, surgery with imatinib therapy; and one patient, endoscopic resection. The tumors preferentially occurred in the lower and middle parts of the thoracic esophagus, with a median size of 36.5 mm. Neoadjuvant and adjuvant imatinib therapies were performed in seven and eight patients, respectively. Administration of imatinib 400 mg/day was the standard regimen. Postoperative follow-up observations were conducted mostly via computed tomography (CT) scans every 3 or 6 months until 5 years after surgery. The tumors recurred in ten patients within 5 years postoperatively (high risk, 38.5%; intermediate risk, 20%; low risk, 0%; very low risk, 0%; three cases of relapse with an unknown risk assessment). A patient with a high-risk GIST died from the tumor 54 months after surgery.
Conclusions: This nationwide survey revealed the current status of esophageal GISTs in Japan and provided important information for making a consensus on the treatment and follow-up method.
Keywords: Esophagus; Gastrointestinal stromal tumor; Nationwide survey.