Clinical Gastroenterology Vol.20 No.9(6)

Theme GIST (gastrointestinal stromal tumor)
Title Extra-gastrointestinal Stromal Tumor (EGIST)
Publish Date 2005/08
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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