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Article Abstract

Gastrointestinal stromal tumors (GISTs) account for approximately 3% of gastrointestinal tumors and are the most prevalent mesenchymal tumors, primarily affecting the stomach and small intestine. In pediatrics, they are considered rare tumors and differ from GISTs that are found in adults because they often lack mutations in the KIT or platelet-derived growth factor α (PDGFRA) genes and are thereby classified as "wild-type." Currently, there are few specific guidelines for treating this type of tumor in the pediatric population. More data are needed to better understand GISTs and develop more effective targeted therapies. The present report describes the case of a patient, now an adult, who was diagnosed with a GIST during childhood and experienced several relapses. A therapeutic regimen was devised and implemented, resulting in a favorable outcome.

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http://dx.doi.org/10.1542/peds.2025-070625DOI Listing

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