It is estimated that between 3,000 and 10,000 GISTs are diagnosed in the United States each year.^{1, 2} Median age of patients at GIST diagnosis is between 66 and 69 years. GIST in patients <30 years old is extremely rare.¹ Fewer than half of GIST patients present with localized primary disease.²

GISTs are the most prevalent mesenchymal, or soft-tissue, tumors of the gastrointestinal tract, occurring most commonly in the stomach (50%) and small bowel (25%).¹ Small-intestinal GISTs tend to be more aggressive than gastric GISTs of equal size.¹

Advanced GIST is characterized by liver metastases and/or dissemination within the abdominal cavity. Lymph node metastases and dissemination to the lungs are both extremely uncommon.¹

Prior to the advent of TKI therapies in GIST, disease-specific median survival was as follows:

Recurrence is common; the NCCN estimates that the median time to recurrence after resection of a high-risk GIST is approximately 2 years.⁴

True local recurrence (limited to the original surgical site) is unusual with GIST. First recurrence usually is within the abdomen and involves the peritoneum, liver, or both. Typically there is also widespread intraperitoneal recurrence.¹

Current goals for the clinical management of GIST are prolonging survival, reducing recurrent disease, optimizing surgery timing and organ preservation, and pharmacologic debulking to improve tumor resectability.¹

Effective management requires a multidisciplinary team encompassing pathology, medical oncology, surgical oncology, and imaging expertise in sarcoma or tumors of the GI tract.¹

Treatment options for GIST include surgery, which continues to be the cornerstone of treatment, and drug therapy using tyrosine kinase inhibitors (TKIs). TKIs have become important treatment options in neoadjuvant and adjuvant settings, as well as in unresectable or metastatic disease. Cytotoxic chemotherapy, on the other hand, is generally considered to be ineffective in the treatment of GIST.¹