appraising treatment for gastrointestinal stromal tumors of the wild-type mutation in the setting of nf1: a case report

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.