Previously known as intestinal neurofibromatosis/neurofibromatosis 3b (INF/NF3b), this disease is now classified as a familial gastrointestinal stromal tumor (GIST) due to its genetic makeup, which was identified when GIST was the sole PDGFRA-mutant tumor found in the gastrointestinal tract. This evidence concerns the gene PDGFRA and neoplasm.