Theme |
Gastrointesinal Submucosal Tumor -- Advances in the Diagnosis and Treatment |
Title |
The c-kit Gene and Gastrointestinal Submucosal Tumors |
Publish Date |
2001/03 |
Author |
Toshirou Nishida |
Department of Surgery E1, Osaka University Graduate School of Medicine |
Author |
Sei-ichi Hirota |
Department of Pathology, Osaka University Graduate School of Medicine |
[ Summary ] |
Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors in the gastrointestinal tract and account for nearly 80% of gastrointes tinal mesenchymal tumors. Myogenic tumors account for 10% to 15%, and the remainders are neurogenic tumors. GISTs express KIT and CD 34 proteins, and show histological similarities to interstitial cells of Cajal (ICCs). Gain-of-function mutations in the c-kit gene cause GISTs and loss-of-function mutations are associated with lack of ICCs and intestinal pacemaker activity, which results in paralytic ileus. The c-kit gene is critical in differentiation and proliferation of ICCs, and mutations in the c-kit gene are an important prognostic factor for patients with gastrointestinal mesenchymal tumors. |