Tomio Arai1, Hiroyuki Kuwano2,3, Tatsuya Miyazaki4, Makoto Sakai2, Makoto Sohda2, Tetsuo Nemoto5, Yasushi Toh6, Yoshihiro Kakeji7, Hisahiro Matsubara8, Yuichiro Doki9. 1. Department of Pathology, Tokyo Metropolitan Geriatric Hospital and Institute of Gerontology, 35-2 Sakaecho, Itabashi-ku, Tokyo, 173-0015, Japan. arai@tmig.or.jp. 2. Department of General Surgical Science, Graduate School of Medicine, Gunma University, Maebashi, Japan. 3. Fukuoka City Hospital, Fukuoka, Japan. 4. Department of Surgery, Japanese Red Cross Maebashi Hospital, Maebashi, Japan. 5. Department of Pathology, Showa University Northern Yokohama Hospital, Yokohama, Japan. 6. Department of Gastroenterological Surgery, National Hospital Organization Kyushu Cancer Center, Fukuoka, Japan. 7. Division of Gastrointestinal Surgery, Department of Surgery, Kobe University Graduate School of Medicine, Kobe, Japan. 8. Department of Frontier Surgery, Graduate School of Medicine, Chiba University, Chiba, Japan. 9. Department of Gastroenterological Surgery, Graduate School of Medicine, Osaka University, Osaka, Japan.
Abstract
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.
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.
Authors: Simon Lott; Michael Schmieder; Benjamin Mayer; Doris Henne-Bruns; Uwe Knippschild; Abbas Agaimy; Matthias Schwab; Klaus Kramer Journal: Am J Cancer Res Date: 2014-12-15 Impact factor: 6.166