Gastrointestinal Stromal Tumor Disease Summary

Last updated: 03 March 2025

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Overview

Gastrointestinal stromal tumors (GIST) are morphologically spindle cell, epithelioid, or occasionally pleomorphic mesenchymal tumors of the gastrointestinal tract as stated in the Introduction section.

GIST are the most common mesenchymal tumors originating from the gastrointestinal tract. There is a high variation in the reported incidence of GIST and details of these are in the Epidemiology section.

GIST are associated with activation mutations in the tyrosine kinase receptor KIT and/or the platelet-derived growth factor receptor-α (PDGFRA). Mechanisms involved in this mutation and development of GIST are in the Pathophysiology section.

History and Physical Examination

The Clinical Presentation section enumerates the presenting symptoms of GIST. Among these symptoms include abdominal pain or discomfort, early satiety and gastrointestinal bleeding.

Diagnosis

Laboratory Tests and Ancillaries and Imaging studies to be considered in the work-up of patients suspected to have GIST are discussed in these sections. It can be noted that biopsy is necessary to confirm the diagnosis of GIST before the initiation of preoperative therapy. Discussion of this procedure is in this section also.

GIST should be differentiated from other gastrointestinal tract tumors and these other types of cancer are enumerated in the Differential Diagnosis section.

Management

Therapeutic options for preoperative or neoadjuvant therapy, chemotherapy and systemic therapy for unresectable, progressive, or metastatic disease are all discussed in the Pharmacological Therapy section. Some of the agents recommended include Imatinib, Pazopanib, Regorafenib, Entrectinib and Larotrectinib.

Some patients may be considered for observation and recommendations on this are enumerated in the Nonpharmacological section.  

Complete surgical excision is the standard treatment of localized or potentially resectable GIST. Indications and surgical options are discussed in detail in the Surgery section.

The Radiation Therapy section discusses the different interventional radiology options for patients with GIST.

The Monitoring section features the parameters to consider in assessing patients being treated for GIST and management of disease progression. 


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