We report herein the case of a 77-year-old woman with a gastrointestinal st
romal tumor (GIST) of the duodenal ampulla. The tumor. which was removed by
pancreatoduodenectomy due to its malignant potential, showed expansive gro
wth in the muscular coat of the duodenal ampulla. Histologically, the tumor
cells were spindle-shaped or round, and exhibited fascicular or storiform
growth pattern with frequent mitotic figures. The immunohistochemical and u
ltrastructural results suggested that the tumor may have originated from th
e interstitial cells of Cajal in the muscular coat of the duodenal ampulla.
To our knowledge, this is the first case of malignant GIST of the duodenal
ampulla to be reported in Japan.