Intraductal papillary mucinous neoplasm of the pancreas associated with neuroendocrine tumor: A case report

INTRODUCTION: Intraductal papillary mucinous neoplasm is an uncommon cystic tumor of pancreas that can be associated with ductal adenocarcinoma. Coexistence of pancreatic IPMN and neuroendocrine tumor is very rare. Here, we report the imaging features of mixed type intraductal papillary mucinous neoplasia of the pancreas with high grade dysplasia together with neuroendocrine carcinoma and perform review of the literature. PRESENTATION: A 68-year old patient has been evaluated for possible IPMN that was suspected during ultrasound. MRI revealed main and side branch duct dilatations. At the head, a contrast enhancing nodular lesion was identified. Due to the presence of high risk stigmata according to guidelines, surgery was performed. Histopathological examination revealed an unusual association, including mixed type IPMN and neuroendocrine carcinoma. DISCUSSION: The concomitant occurrence of pancreatic IPMN and neuroendocrine tumor has been reported in case studies and brief reviews. Yet, the imaging findings and underlying molecular mechanisms of this entity has not been fully understood. In addition to this unusual association, pancreatic intraepithelial neoplasia was also detected in the present case. Although majority of neuroendocrine tumor associated IPMNs were reported to be having low grade dysplasia, our patient had high grade dysplasia. Further studies and reviews with larger groups are needed to establish imaging features and underlying molecular mechanisms of this rare association. CONCLUSION: Although the major concern during work-up of IPMN is presence of associated pancreatic ductal adenocarcinoma, the possibility of neuroendocrine tumor, in the presence of a hypervascular solid foci on imaging studies should be kept in mind.