ABSTRACT
Gastrointestinal stromal tumors (GISTs), the archetypal and commonest mesenchymal neoplasms of the digestive tract, occur most regularly in the stomach. GISTs are a great example of how science can impact outcomes in cancer patients. The discovery by Hirota in 1998 that GIST contained mutations in c-KIT was a breakthrough for advancing the biology and treatment of GIST. The identification of fundamental hallmark of the biology of GIST resulted in the rapid transformation of this treatment paradigm. Small-molecule tyrosine kinase inhibitors have been developed and found to be effective in the treatment of GIST resulting in dramatic improvement in the outcome of patients with this disease. Neurofibromatosis type I is an autosomal dominant disorder occurring in one in 3,000 individuals worldwide. This autosomal dominant syndrome more frequently manifests with tumors of the nervous system. Accurate risk stratification of gastrointestinal stromal tumors has become increasingly important as effective adjuvant therapy emerges.
