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Gastrointestinal Stromal Tumor (GIST)

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Surgical Oncology

Abstract

Gastrointestinal stromal tumors (GISTs) are the most common sarcoma of the GI tract and account for 1–3 % of all GI malignancies. GISTs arise from the interstitial cells of Cajal, an intestinal pacemaker cell located in and around the myenteric plexus. They typically arise in the stomach (65–70 %) or small intestine (25–45 %). An important immunohistochemical marker is KIT, a membrane receptor with tyrosine kinase activity that is present in most GISTs (80–95 %). Some GISTs (5–7 %) have a mutation in platelet-derived growth factor receptor alpha (PDGFRA) instead. Several factors including tumor site, size greater than 5 cm, and greater than 5 mitoses per 50 high-power field predict aggressive behavior and recurrence. The treatment for localized GISTs is resection. Systemic chemotherapy and radiation are ineffective. The tyrosine kinase inhibitor imatinib improves progression-free survival in patients with metastatic disease, and its use in the adjuvant and neoadjuvant settings is increasingly common. Many patients ultimately develop imatinib resistance, and for these patients, dose escalation or the use of another tyrosine kinase inhibitor is recommended.

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Abbreviations

ACOSOG:

American College of Surgeons Oncology Group

ACRIN:

American College of Radiology Imaging Network

AGITG:

Australasian Gastro-Intestinal Trials Group

AJCC:

American Joint Commission on Cancer

CALGB:

Cancer and Leukemia Group B

CT:

Computed tomography

DOG1:

Discovered on GIST 1

ECOG:

Eastern Cooperative Group

EORTC:

European Organization for Research and Treatment of Cancer

ESMO:

European Sarcoma Network Working Group

EUS:

Endoscopic ultrasound

FNA:

Fine-needle aspiration

GIST:

Gastrointestinal stromal tumor

HPF:

High-powered field

ISG:

Italian Sarcoma Group

MRI:

Magnetic resonance imaging

NCCN:

National Comprehensive Cancer Network

OS:

Overall survival

PDGFRA:

Platelet-derived growth factor receptor alpha

PET:

Positron emission tomography

RFS:

Recurrence-free survival

SCF:

Stem cell factor

SSG:

Scandinavian Sarcoma Group

SWOG:

Southwest Oncology Group

TKI:

Tyrosine kinase inhibitor

TNM:

Tumor/node/metastasis

UICC:

International Union Against Cancer

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Author information

Authors and Affiliations

  1. Department of Surgery, Naval Hospital Jacksonville, 2080 Child Street, Jacksonville, 32214, FL, USA

    Lesly A. Dossett M.D., M.P.H.

  2. Department of Surgical Oncology and Endocrine Surgery, Vanderbilt University Medical Center, 2220 Pierce Ave 597 PRB, Nashville, 37232, TN, USA

    Nipun B. Merchant M.D., F.A.C.S.

Authors
  1. Lesly A. Dossett M.D., M.P.H.
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  2. Nipun B. Merchant M.D., F.A.C.S.
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Corresponding author

Correspondence to Nipun B. Merchant M.D., F.A.C.S. .

Editor information

Editors and Affiliations

  1. Department of Surgery, LSU Health Sciences Center-Shreveport, Shreveport, Louisiana, USA

    Quyen D. Chu

  2. Department of Surgery, Jersey Shore University Medical Center, Neptune, New Jersey, USA

    John F. Gibbs

  3. John C. McDonald Regional Transplant Center, Willis Knighton Health System, Shreveport, Louisiana, USA

    Gazi B. Zibari

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Dossett, L.A., Merchant, N.B. (2015). Gastrointestinal Stromal Tumor (GIST). In: Chu, Q., Gibbs, J., Zibari, G. (eds) Surgical Oncology. Springer, New York, NY. https://doi.org/10.1007/978-1-4939-1423-4_28

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  • DOI: https://doi.org/10.1007/978-1-4939-1423-4_28

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  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-1-4939-1422-7

  • Online ISBN: 978-1-4939-1423-4

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