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Table 2 Comparison of characteristics of GIST in NF1 and sporadic cases

From: Rare triad of periampullary carcinoid, duodenal gastrointestinal stromal tumor and plexiform neurofibroma at hepatic hilum in neurofibromatosis type 1: a case report

Characteristics GIST in NF1 Sporadic GIST
Most common location Small bowel Stomach
Solitary or multiple Multiple Solitary
Association with other gastrointestinal tumors like carcinoid Frequent Rare
KIT or PDGFRA mutations Absent Present
Probable molecular pathogenetic mechanism Activation of ras-MAP kinase cascade
Mitotic recombination
Loss of heterozygosity at 14q and 22q
Gain of function mutation of c-kit proto-oncogene
Response to Imatinib mesylate Poor Good