The intraductal growth of nonfunctioning pancreatic endocrine tumors (NFPTs) is been considered to be rare, and in our survey of the English literature only three cases have been described previously. We herein report the case of a 36-year-old male with a malignant NFPT that uniquely grew within the lumen of the main pancreatic duct (MPD) and completely obstructed the MPD by endoscopic retrograde pancreatography (ERP). Endoscopic ultrasonography clearly detected the tumor with intraductal growth. In addition, positron emission tomography using 18F-fluorodeoxyglucose (FDG) and CT by the same scanner (FDG-PET/CT) showed an enhanced uptake of FDG in the tumor. A pylorus-preserving pancreaticoduodenectomy and regional lymphadenectomy were performed under a preoperative diagnosis of a NFPT. Microscopically, positive immunoreactions for synaptophisin and vasoactive intestinal peptide indicated neuroendocrine differentiation of the tumor while in addition metastasis to a lymph node along the common hepatic artery was also observed. The patient has survived for six months after surgery without any evidence of recurrence and metastasis. Both ERP and FDG-PET/CT were thus found to be useful for predicting the malignant potential of NFPTs in the preoperative diagnosis.
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