Abstract
Patients with multiple endocrine neoplasia type 1 (MEN1) are at high risk of malignant pancreatic neuroendocrine tumors (pNETs). Structural imaging is typically used to screen for pNETs but is suboptimal for stratifying malignant potential. To determine the utility of fluorodeoxyglucose (18F) positron emission tomography/computed tomography (18F-FDG PET/CT) for predicting the malignant potential of pNETs in MEN1. Retrospective observational study. Tertiary referral hospital. Forty-nine adult patients with MEN1 carrying a common MEN1 mutation who underwent 18F-FDG PET/CT for MEN1 surveillance between 1 January 2010 and 30 September 2016. Structural and functional imaging (magnetic resonance imaging, CT, ultrasonography, and 18F-FDG PET/CT) and surgical histopathology. pNET size, behavior, and histopathology. Twenty-five (51.0%) of 49 patients studied had pancreatic lesions on structural imaging. Five (25%) of these had 18F-FDG-PET-avid lesions. In addition, two had solitary FDG-avid liver lesions, and one a pancreatic focus without structural correlate. Eight patients with pNETs underwent surgery (three FDG-avid lesions and five nonavid pNETs). The Ki-67 index was ≥5% in FDG-avid pNETs and <2% in nonavid pNETs. Overall, six of the eight (75%) patients with FDG-avid hepatopancreatic lesions harbored aggressive or metastatic NETs compared with one of 41 patients (2.4%) without hepatopancreatic FDG avidity [P < 0.001; sensitivity; 85.7% (95% confidence interval [CI], 48.7% to 99.3%); specificity, 95.2% (95% CI, 84.2% to 99.2%)]. 18F-FDG PET/CT is an effective screening modality in MEN1 for identifying pNETs of increased malignant potential. Surgical resection is recommended for FDG-avid pNETs.
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