Advanced gastrointestinal stromal tumors (GISTs): A short review
Letissier, Octave
Advanced gastrointestinal stromal tumors (GISTs): A short review - 2025.
77
The management of advanced gastrointestinal stromal tumors (GISTs) has progressed in recent years, as highlighted by the 2024 update of the Thésaurus National de Cancérologie Digestive (TNCD). Biopsy or circulating tumor DNA (ctDNA) testing for mutations in KIT and PDGFRA is essential. Imatinib remains the cornerstone of treatment in advanced GIST with KIT mutation, at the standard dose of 400 mg/d or 800 mg/d in the presence of exon 9 mutation. Secondary surgical resection may be considered after 6 to 12 months of imatinib in unresectable non-metastatic GIST. After progression on imatinib 400 mg/d, and after checking for compliance and potential drug interactions, an imatinibemia measurement and ctDNA analysis are used to rule out imatinib underdosing and to look for imatinib-resistant mutations in KIT. Options include increasing the dose to 800 mg/d or switching to a different tyrosine kinase inhibitor, with sunitinib as a priority. Regorafenib and ripretinib are the standard-of-care third- and fourth-line treatments respectively. Inoperable or metastatic GISTs with PDGFRA p.D842V mutation should be treated with avapritinib. GISTs without KIT or PDGFRA mutations are often slow-growing, and destruction or excision of metastases may be indicated. Some specific GISTs (NTRK fusion, BRAFV600E mutation, etc.) may benefit from targeted treatments. Whatever the line of treatment, it is advisable to ask a NETSARC network center for information on ongoing or future trials.
Advanced gastrointestinal stromal tumors (GISTs): A short review - 2025.
77
The management of advanced gastrointestinal stromal tumors (GISTs) has progressed in recent years, as highlighted by the 2024 update of the Thésaurus National de Cancérologie Digestive (TNCD). Biopsy or circulating tumor DNA (ctDNA) testing for mutations in KIT and PDGFRA is essential. Imatinib remains the cornerstone of treatment in advanced GIST with KIT mutation, at the standard dose of 400 mg/d or 800 mg/d in the presence of exon 9 mutation. Secondary surgical resection may be considered after 6 to 12 months of imatinib in unresectable non-metastatic GIST. After progression on imatinib 400 mg/d, and after checking for compliance and potential drug interactions, an imatinibemia measurement and ctDNA analysis are used to rule out imatinib underdosing and to look for imatinib-resistant mutations in KIT. Options include increasing the dose to 800 mg/d or switching to a different tyrosine kinase inhibitor, with sunitinib as a priority. Regorafenib and ripretinib are the standard-of-care third- and fourth-line treatments respectively. Inoperable or metastatic GISTs with PDGFRA p.D842V mutation should be treated with avapritinib. GISTs without KIT or PDGFRA mutations are often slow-growing, and destruction or excision of metastases may be indicated. Some specific GISTs (NTRK fusion, BRAFV600E mutation, etc.) may benefit from targeted treatments. Whatever the line of treatment, it is advisable to ask a NETSARC network center for information on ongoing or future trials.
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