Molekularpathologisch determinierte multimodale Therapie gastrointestinaler Stromatumoren

Abstract

BackgroundGastrointestinal stromal tumors (GIST) comprise a broad spectrum of different molecular subtypes. Due to improved accessibility to molecular pathology, genotyping has become an integral part of interdisciplinary case discussions.AimTo provide an overview on existing literature on the relevance of genotyping for treatment decisions in GIST.Results and conclusionsMost patients with KIT exon 11 mutations and high risk of relapse benefit from adjuvant imatinib treatment. The evidence for this benefit is weak for less frequent KIT genotypes (e.g., exon 9, 13, and 17 mutations). Neoadjuvant treatment is particularly relevant in imatinib-sensitive subtypes when significant surgery-related morbidity is expected. For patients in the metastatic setting, multimodal treatment can be considered in responding patients when macroscopically complete resection seems feasible. Next generation inhibitors such as ripretinib in pretreated GIST and particularly avapritinib in GIST with PDGFRA (platelet-derived growth factor receptor A) mutations are associated with relevant tumor regression. The role for multimodal approaches in the respective treatment settings remains to be determined.