Latest Advances in the Management of Pediatric Gastrointestinal Stromal Tumors

SIMPLE SUMMARY: Though gastrointestinal stromal tumor is the most common mesenchymal neoplasm of the gastrointestinal tract, it is a rare entity among pediatric patients. It is usually characterized by a different molecular biology, histology and clinical course. Therefore, different handling of pediatric GIST is needed. Herein, we review the latest updates to the management of pediatric gastrointestinal tumors with a particular focus on the advances in molecular biology of the disease and emerging treatment with kinase inhibitors that could serve as targeted therapy. ABSTRACT: Gastrointestinal stromal tumor is the most common mesenchymal neoplasm of the gastrointestinal tract, usually found in elderly adults. It is infrequent among pediatric patients and usually differs biologically from adult-type diseases presenting mutations of KIT and PDGFR genes. In this population, more frequent is the wild-type GIST possessing SDH, TRK, RAS, NF1 mutations, among others. Both tumor types require individualized treatment with kinase inhibitors that are still being tested in the pediatric population due to the different neoplasm biology. We review the latest updates to the management of pediatric gastrointestinal tumors with a particular focus on the advances in molecular biology of the disease that enables the definition of possible resistance. Emerging treatment with kinase inhibitors that could serve as targeted therapy is discussed, especially with multikinase inhibitors of higher generation, the effectiveness of which has already been confirmed in the adult population.