GEIS guidelines for gastrointestinal sarcomas (GIST)

Area of Science:

• Oncology
• Molecular Biology
• Gastroenterology

Background:

• Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal neoplasms of the digestive tract.
• GISTs are characterized by specific morphology and CD117 (c-kit) positivity, often driven by KIT or PDGFRA gene mutations.
• Rarely, GISTs can arise in extravisceral sites, including the omentum, mesentery, pelvis, and retroperitoneum.

Purpose of the Study:

• To highlight the multidisciplinary approach in understanding and treating GISTs.
• To emphasize the impact of targeted therapy on patient survival.
• To review the current landscape of targeted agents for metastatic GIST.

Main Methods:

• Review of GIST characteristics, including morphology and genetic drivers.
• Analysis of the role of multidisciplinary teams in advancing GIST management.
• Evaluation of the clinical impact of targeted therapies such as imatinib, sunitinib, and regorafenib.

Main Results:

• Imatinib established targeted therapy as the first effective molecular treatment for solid tumors.
• Median survival for advanced or metastatic GIST patients increased from 18 to over 60 months after imatinib introduction.
• Sunitinib and Regorafenib are approved for second- and third-line treatments in metastatic GIST, respectively.

Conclusions:

• GISTs serve as a paradigm for successful multidisciplinary oncology care and targeted therapy development.
• Targeted therapies have dramatically improved outcomes for patients with advanced and metastatic GIST.
• Continued research and collaboration are crucial for further advancements in GIST treatment.