Advances in the management of gastrointestinal stromal tumors (GISTs)

Gastrointestinal stromal tumors are rare neoplasms developing from cells of Cajal in the gastrointestinal tract. The mainstay of such tumors treatment is surgery, whenever possible. The therapeutic management of inoperable and metastatic disease is based on tyrosine kinase inhibitors and imatinib is the main drug recommended for first line treatment. The introduction of imatinib and other inhibitors improved survival outcomes for this disease, but due to primary and secondary resistance there is still the urgent need for new medications. This paper presents the progress in the systemic therapy of GISTs based on the latest scientific data. The newly developed agents (ripretinib, avapritinib) meet the need to treat patients after the failure of previously available therapies and those with PDGFRA mutation D842V associated with resistance to imatinib