Gastrointestinal Stromal Tumor (GIST): Diagnosis and Treatment

  • Attila Kollár
  • Pedro Nazareth AguiarJr.
  • Nora Manoukian Forones
  • Ramon Andrade De MelloEmail author


Gastrointestinal stromal tumor is the most common mesenchymal neoplasm arising the gastrointestinal tract. The primary tumor ist most common in the stomach (60–70%), followed by the small intestine (20–25%), colon and rectum (5%), and esophagus (less than 5%). The median age at diagnosis is between 60 and 65 years. Histologically, GIST is characterized by its immunopositivity for CD117 (KIT). Clinically, there is a paucity of specific symptoms and a majority of cases becomes symptomatic after local compression caused by tumor mass. Surgery is the main treatment for localized disease. The indication for adjuvant imatinib is based upon risk factors such as primary tumor site, tumor size and number of mitosis. KIT-targeted tyrosine kinase inhibitors (TKI) are the cornerstone for the treatment of metastatic disease. Imatinib is the drug of choice in the first-line setting. Sunitinib, regorafenib, and pazopanib are studied further-line treatment optionse. Immunotherapy studies are ongoing for TKI-refractory patients.


Gastrointestinal stromal tumors Molecular targeted therapy Surgery 



Gastrointestinal Stromal Tumor


Platelet-derived Growth Factor Receptor Alpha


Neurofibromatosis type I


Succinate Dehydrogenase Complex Subunit B, C or D


Receptor Tyrosine Kinase


Smooth Muscle Actin


Stem Cell Factor


Discovered on GIST


Succinate Dehydrogenase


European Society for Medical Oncology


Computed Tomography


Magnetic Resonance Image


Positron Emission Tomography


Armed Forced Institute of Pathology


National Institutes of Health


High Power Fields


Not Available


Endoscopic Ultrasound


Relapse-free Survival


Scandinavian Sarcoma Group


Confidence Internal


Adenosine Triphosphate


National Cancer Comprehensive Network


Thyroid-Stimulating Hormone


Best Supportive Care


Tumor-associated macrophages


Cytotoxic T-lymphocyte Associated Protein 4


Programmed-death Receptor 1


Programmed-death Receptor Ligand 1




Response Evaluation Criteria In Solid Tumors


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Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Attila Kollár
    • 1
  • Pedro Nazareth AguiarJr.
    • 2
  • Nora Manoukian Forones
    • 3
  • Ramon Andrade De Mello
    • 4
    • 5
    Email author
  1. 1.Department of Medical OncologyInselspital, University of BernBernSwitzerland
  2. 2.Faculdade de Medicina do ABCSanto AndréBrazil
  3. 3.Escola Paulista de Medicina, Federal University of São Paulo (UNIFESP)São PauloBrazil
  4. 4.Division of Medical OncologyFederal University of São Paulo (UNIFESP) & Hospital Israelita Albert EinsteinSão PauloBrazil
  5. 5.Department of Biomedical Sciences and MedicineUniversity of AlgarveFaroPortugal

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