Gastrointestinal stromal tumors (GISTs) are a specific pathological entity of mesenchymal neoplasia discovered in the 1980s. These tumors originate from the interstitial cells of Cajal and represent the most prevalent type of mesenchymal tumor of the gastrointestinal tract. GISTs are clinically indistinguishable from other digestive tumor groups, and their pretherapeutic investigation is facilitated by digestive endoscopy and current imaging techniques such as injected CT, MRI, and echo-endoscopy that allows tissue biopsy. In addition, molecular genetics testing and pathological evaluation are critical for diagnostic, predictive, and prognostic evaluation. Currently, surgery remains the cornerstone of treatment, but the therapeutic arsenal has been enriched since the discovery of anti-tyrosine kinase receptor targeted therapies. Despite current advances, a unique prognostic hierarchy for each patient is essential to guide them through their therapeutic journey. A good understanding of the biological mechanisms specific to GISTs will allow us to develop new therapies in the future to improve the prognosis of patients.
What is already known about the topic?
Gastrointestinal stromal tumors are a subgroup of neoplasia involving the mesenchyme of the gastrointestinal tract. Their onco-surgical management currently relies on the synergy of surgery and tyrosine kinase inhibitors.
What does this article bring up for us?
The purpose of this article is to introduce this nosological entity to non-specialists and specialists so that they can better understand this neoplasia, which is often overlooked despite being the most common mesenchymal tumor of the digestive tract. A clinical case is presented, followed by a discussion of current recommendations. In addition, the role of the anatomopathologist and clinical geneticist in the oncological management is highlighted.
Keywords
Gastrointestinal stromal tumors, GIST, mesenchymal tumors, oncology, targeted therapy