대한흉부영상의학회 Weekly Chest Cases

Case No : 256 Date 2002-09-19

Courtesy of Chin A Yi, M.D. Tae Sung Kim, M.D., Kyung Soo Lee, M.D. / Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
Age/Sex 63 / F
Chief ComplaintDyspnea for several days

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Diagnosis With Brief Discussion

Diagnosis: Malignant Gastrointestinal Stormal Tumor (GIST) of the Esophagus
Radiologic Findings: About 6.5x4 cm sized well-encapsulated homogeneous intramural mass is seen involving 8 cm segment of distal esophagus. Ivor Lewis operation was done due to frozen biopsy from the mass show spindle cell tumor with atypia.
Brief Review: Although rare elsewhere in the gastrointestinal tract, leiomyomas (LMs) are the most common esophageal mesenchymal neoplasms. In contrast, gastrointestinal stromal tumors (GISTs) predominate in the stomach and intestines but have not been documented in the esophagus. There is a study to determine the clinicopathologic features and frequency of esophageal GISTs compared with LMs and leiomyosarcomas (LMSs) of the esophagus. A total of 68 stromal/smooth muscle tumors from the Armed Forces Institute of Pathology and the Haartman Institute of University ofHelsinki were reclassified by current histologic and immunohistochemical criteria. There were 17 GISTs, 48 LMs, and three LMSs. The esophageal GISTs occurred in 12 men and five women with a median age of 63 years (range, 49-75 years). All tumors were from the lowest third of the esophagus, and the most common complaint was dysphagia, whereas two tumors were detected incidentally. All LMs were clinically indolent tumors with no tumor-related mortality. All three LMSs were large high-grade tumors that showed muscle cell markers but no CD117. All patients died of disease. Esophageal GISTs showed mutations in exon 11 of c-kit as described previously in gastric and intestinal GISTs. The separation of GISTs from esophageal LMs is important diagnostically because the former group has a high risk of malignant behavior.
References: 1. Miettinen M et al. Esophageal stromal tumors: a clinicopathologic, immunohistochemical, and molecular genetic study of 17 cases and comparison with esophageal leiomyomas and leiomyosarcomas. Am J Surg Pathol. 2000 Feb;24(2):211-22.
Keywords: Esophagus, Malignant tumor, Malignant Gastrointestinal Stormal Tumor (GIST) of the Esophagus

No. of Applicants : 20

▶ Correct Answer : 8/20, 40.0%

- Annecy Hospital, France Genin Gilles
- Chung-Ang University Hospital, Korea Jae Hyung Park
- Ewha Womans' University, Mokdong Hospital, Korea Sung Shine Shim
- Gospel Hospital Kosin University, Korea Kyung Hwa Jung
- Gwangmoung Seongae Hospital, Korea Jiyong Rhee
- Hangang Sacred Heart Hospital, Korea Eil Seong Lee
- Planned Parenthood Federation of Korea Kyu Sung Kwack
- Seoul National University Hospital, Korea Jung-Gi Im

▶ Semi-Correct Answer : 12/20, 60.0%

- Aeromedical Center, Sacheon Air-base, Korea Seung Soo Lee
- Camerino Hospital, Italy Roberto Evangelisti
- Choong-ju Hospital Konkuk University, Korea Chang Hee Lee
- CHU Nancy-Brabois, France Denis Regent
- Kangdong Sacred Heart Hospital, Korea Young Cheol Yoon
- MH[CTC] Pune, India Vivek Sharma
- Ondokuz Mayis Univercity, Samsun, Turkey Cetin Celenk
- Ospedale di Jesi, Italy Giancarlo Passarini
- Sao Camilo Clinic, Brazil Arismar Leon Pereira
- Stedelijk OLV Ziekenhuis Mechelen, Belgium Ivan Pilate
- Uiwang SUN Hospital, Korea Ki Taek Hong
- Xray Clinic, Pune, India M D Rahalkar

대한흉부영상의학회 Korean Society of Thoracic Radiology

Archive of Old Cases

Case No : 256 Date 2002-09-19

Discussion

No. of Applicants : 20