Gastrointestinal Stromal Tumors

Gastrointestinal stromal tumors are rare malignancies involving the intestinal tract. They are now easily recognized using standard immunohistochemistry. Management in the past was primarily surgical until oncogenic driver mutations in the KIT and PDGFRA receptors were identified leading to the standard use of tyrosine kinase inhibitors in the adjuvant, neoadjuvant, and metastatic disease setting. While the majority of patients benefit from these approaches, intrinsic tumor resistance from specific KIT and PDGFRA mutations, as well as other oncogenic divers, requires a complete understanding of the molecular drivers of each tumor for optimal management of disease. In addition, the development of secondary resistance in patients with metastatic disease is now managed with multimodality therapies for optimal long-term tumor control.