Tehran University of Medical Sciences International Journal of Hematology-Oncology and Stem Cell Research 2008-2207 19 3 2025 07 21 Appraising Treatment for Gastrointestinal Stromal Tumors of the Wild-Type Mutation in the Setting of NF1: A Case Report 296 302 EN Darshan Gundala Division of Surgical Oncology, Department of Surgery, Northwestern University Feinberg School of Medicine, Chicago, IL, United States Rachel Joung Division of Surgical Oncology, Department of Surgery, Northwestern University Feinberg School of Medicine, Chicago, IL, United States Amy Wells Northwestern Medicine Cancer Centers, Northwestern Medicine Regional Medical Group, Winfield, IL, United States Ashley Hardy Northwestern Medicine Cancer Centers, Northwestern Medicine Regional Medical Group, Winfield, IL, United States John Abad 1) Division of Surgical Oncology, Department of Surgery, Northwestern University Feinberg School of Medicine, Chicago, IL, United States 2) Northwestern Medicine Cancer Centers, Northwestern Medicine Regional Medical Group, Winfield, IL, United States Akhil Chawla 1) Division of Surgical Oncology, Department of Surgery, Northwestern University Feinberg School of Medicine, Chicago, IL, United States 2) Northwestern Medicine Cancer Centers, Northwestern Medicine Regional Medical Group, Winfield, IL, United States 3) Robert H. Lurie Comprehensive Cancer Center of Northwestern University, Chicago, IL, United States 2024 05 14 2024 10 12 Gastrointestinal stromal tumors (GISTs) are rare mesenchymal neoplasms that can occur anywhere in the GI tract, but commonly present in the stomach and small bowel. Here we present a 39-year-old male with a suspected GIST in the setting of type I neurofibromatosis (NF1). Given the history of NF1 and presumable insensitivity to Imatinib, surgical oncology opted for resection of the mass after discussion with the multidisciplinary sarcoma tumor board. Instead of neoadjuvant therapeutic options for wild-type (WT) NF1-related GIST, surgical resection remains the most advantageous treatment. The efficacy of tyrosine kinase inhibition and other chemotherapies tailored for WT GIST is currently untenable and warrants increased clinical trials and exploration of WT pathogenesis concerning NF1 to support Imatinib-sensitive patients. https://ijhoscr.tums.ac.ir/index.php/ijhoscr/article/view/2240 https://ijhoscr.tums.ac.ir/index.php/ijhoscr/article/download/2240/1095