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

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

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

ISSN: 1471-2407