Endocrine Tumor of the Pancreas With Intraductal Spreading

Primary neuroendocrine tumors of the main pancreatic duct: a rare entity

“…In this situation, the clinical presentation and the pathological features are usually very different from those associated with our cases: (a) the clinical picture is usually severe, with symptomatic acute or chronic pancreatitis; (b) the endocrine tumor is usually large; (c) its histological and morphological characteristics lie within the spectrum of conventional pancreatic neuroendocrine tumors; (d) direct infiltration of the excretory duct by tumor cells is not constant; when it occurs, it clearly results from the entrapment of the excretory duct by a primarily intra-parenchymal tumor [5]; (e) most of these tumors behave as malignant tumors and present with lymph node and/or distant metastases at diagnosis [21]. The second situation is represented by the rare cases of pancreatic endocrine tumors with intra-ductal growth [22][23][24][25][26][27][28][29][30][31][32], defined by the presence of a large intraluminal tumor mass, obliterating the lumen of an excretory duct, which may be isolated or, more commonly, connected to an adjacent intra-parenchymal mass. Any of our cases was associated with an intra-luminal component.…”