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Despite the increased incidence of pancreatic cancer, reported data of collision pancreatic tumors are very rare, limited just to sporadic cases. There are only two described cases of the collision pancreatic tumor consisting of neuroendocrine and pancreatic ductal adenocarcinoma in the literature. Currently, we are presenting a case of a young female patient with pancreatic ductal adenocarcinoma surrounding a smaller focal lesion of the well-differentiated neuroendocrine pancreatic tumor. The patient underwent proximal pancreaticoduodenectomy with uneventful postoperative course. Histogenesis of these colliding tumors remains unclear. However, there are several proposed theories. Surgical resection could be the treatment of choice of resectable cases; however, preoperative diagnosis is virtually impossible.

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