Multifocal gastrointestinal stromal tumors (GISTs) of the small intestine in patients with neurofibromatosis type 1 (NF-1): Meta-analysis and systematic review of the literature

This meta-analysis evaluates small intestinal GIST (SI-GIST) prevalence in patients with neurofibromatosis type 1 (NF-1), revealing a profound oncogenic propensity and markedly increased prevalence compared to the general population. Tumors are often multifocal (54%), solitary SI-GIST (41%), or solitary duodenal (5%). The study aims to improve clinical surveillance and NF-1 management, emphasizing the need for heightened clinical suspicion in this high-risk population due to extraordinary disease susceptibility.