Abstract

Context Intraductal papillary mucinous neoplasms (IPMNs) are uncommon tumours which can be associated withpancreatic and extrapancreatic malignancies. The association of IPMN and neuroendocrine tumours of the pancreas has been reported previously but is exceedingly rare. Case report We report three cases of IPMN treated with totalpancreatectomy/extended distal pancreatectomy. Histopathological analysis of the resected specimens revealedconcomitant IPMN and neuroendocrine tumour. Two patients had adenocarcinoma as well. Conclusions The presence of anIPMN may place the entire pancreas at risk of developing other tumour types and vigilance during all stages of managementis necessary to ensure optimal treatment.