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Review
. 2018 Nov 15:3:92.
doi: 10.21037/tgh.2018.10.13. eCollection 2018.

Approach to wild-type gastrointestinal stromal tumors

Joshua K Kays  1 , Jeffrey D Sohn  2 , Bradford J Kim  1 , Katherine Goze  1 , Leonidas G Koniaris  1
Affiliations
Review

Approach to wild-type gastrointestinal stromal tumors

Joshua K Kays et al. Transl Gastroenterol Hepatol. .

Abstract

Gastrointestinal stromal tumors (GISTs) arise from the intestinal pacemaker cells of Cajal. Wild-type gastrointestinal stromal tumors (WT-GIST) are a unique and uncommon subtype of GISTs that lack activating mutations in the tyrosine kinase c-KIT or platelet derived growth factor receptor alpha (PDGFRA) receptors. The lack of these growth-stimulating mutations renders tyrosine kinase receptor inhibitors, such as imatinib mesylate, relatively ineffective against these tumors. WT-GIST arises most commonly due to underlying alternate proliferative signals associated with germ-line, genetic mutations. WT-GIST frequently arises in patients with BRAF mutations, Carney's Triad or neurofibromatosis type-1 (NF-1). All patients with WT-GIST require a careful examination for germ-line mutations and very close observation for recurrent tumors. Surgery remains a mainstay therapy for these patients. This review aims to discuss the most recent data available on the diagnosis and treatment of WT-GIST.

Keywords: Gastrointestinal stromal tumor (GIST); sarcoma; succinate dehydrogenase (SDH); wild-type gastrointestinal stromal tumor (WT-GIST).

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Conflict of interest statement

Conflicts of Interest: The authors have no conflicts of interest to declare.

Figures

Figure 1
Figure 1
Diagnostic algorithm for WT-GIST; WT-GIST, wild-type gastrointestinal stromal tumor.
Figure 2
Figure 2
Treatment algorithm for WT-GIST. WT-GIST, wild-type gastrointestinal stromal tumor.
See this image and copyright information in PMC

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