臨牀消化器内科 Vol.20 No.9(6)


特集名 GIST (gastrointestinal stromal tumor)
題名 消化管外に発生するGIST (extra-gastrointestinal stromal tumor ; EGIST)
発刊年月 2005年 08月
著者 櫻井 信司 群馬大学医学部応用腫瘍病理
著者 佐久間 裕司 神奈川県立がんセンター臨床研究所がん分子病態研究部門
【 要旨 】 近年,GISTは,力ハール細胞 (ICC) 由来,ないしICCへの分化を示す腫瘍と考えられるようになり,その大部分はICCの存在する消化管固有筋層に連続して発生する.しかし,ごくまれに大網,腸間膜など,消化管以外の部位に発生するGISTが報告され,EGISTと呼ばれるようになった.ICCの存在しない,消化管外に発生することから,GISTのICC由来を疑問視する意見もある.しかし,大網にはKIT陽性を示すICC様細胞が存在し,また,腹部以外の臓器に発生したEGISTの報告は今までにない.GISTはどこにでも発生する腫瘍ではなく,現時点で,“間葉系幹細胞由来の腫瘍”と曖昧に定義することは,診断,治療を考えるうえで適切でない.
Theme GIST (gastrointestinal stromal tumor)
Title Extra-gastrointestinal Stromal Tumor (EGIST)
Author Shinji Sakurai Department of Tumor Pathology, Gunma University Graduate School of Medicine
Author Yuji Sakuma Molecular Pathology and Genetic Division, Kanagawa Cancer Center Research Institute
[ Summary ] Gastrointestinal stromal tumors (GIST) are currently considered to derive from interstitial cells of Cajal (ICC). However, there have been some reports that mesenchymal tumors, primarily in extra-gastrointestinal sites, such as the omentum, the mesentery, the pelvic cavity, the liver and the retroperitoneum, were also immunoreactive for KIT. Some of these showed c-kit gene mutations. However KIT-positive ICC have not been observed in these sites. These tumors are known as extra-gastrointestinal stromal tumors (EGIST) and the development of EGIST cast doubt on ICCs as the origin of GIST. However, we discovered KIT positive ICC-like cells just beneath mesothelial cells in the omentum. Moreover, the primary sites for EGIST in previous reports were limited to the abdominal region. These results indicate that KIT-positive bipolar mesenchymal cells may be counterparts of ICCs in the GI tract and EGIST may develop from those ICC-like cells. Further studies will be needed to understand the developmental origins of EGIST.
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