A coexisting of intraductal papillary mucinous hyperplasia (IPMH) and islet
cell tumor with nesidioblastosis of the pancreas in a 51-year-old man is r
eported. All of the clinical data indicated an insulinoma. A distal pancrea
tectomy was performed. A discrete mass measuring 1.9 x 2.0 cm was grossly i
dentified in the tail of the pancreas. There were no other gross lesions. A
n islet cell tumor with nesidioblastosis was confirmed by immunostains and
ultrastructural study. in addition, an IPMH was found that involved mainly
branches of the pancreatic duct. The islet cell tumor and IPMH were topogra
phically separated; however, there was a histologically intimate relationsh
ip between the nesidioblastosis and the IPMH. These findings indicate that
the IPMH may have derived from autocrine and paracrine influences on the ex
isting duct epithelial cells. To the best of our knowledge, this is the fir
st report of nesidioblastosis coexisting with islet cell tumor and IPMH.