Abstract
PurposeFocal congenital hyperinsulinism (CHI) is curable by surgery, which is why identification of the focal lesion is crucial. We aimed to determine the use of 18F–fluoro-dihydroxyphenylalanine (18F-DOPA) PET/CT vs. 68Ga-1,4,7,10-tetraazacyclododecane-1,4,7,10-tetraacetic-acid-1-Nal3-octreotide (68Ga-DOTANOC) PET/CT as diagnostic tools in focal CHI.MethodsPET/CT scans of children with CHI admitted to Odense University Hospital between August 2005 and June 2016 were retrospectively evaluated visually and by their maximal standardized uptake values (SUVmax) by two independent examiners, blinded for clinical, surgical and pathological data. Pancreatic histology was used as the gold standard. For patients without surgery, the genetic profile served as the gold standard.ResultsFifty-five CHI patients were examined by PET/CT (18F-DOPA n = 53, 68Ga-DOTANOC n = 18). Surgery was performed in 34 patients, no surgery in 21 patients. Fifty-one patients had a classifiable outcome, either by histology (n = 33, 22 focal lesions, 11 non-focal) or by genetics (n = 18, all non-focal). The predictive performance of 18F-DOPA PET/CT to identify focal CHI was identical by visual- and cut-off-based evaluation: sensitivity (95% CI) of 1 (0.85–1); specificity of 0.96 (0.82–0.99). The optimal 18F-DOPA PET SUVmax ratio cut-off was 1.44 and the optimal 68Ga-DOTANOC PET SUVmax cut-off was 6.77 g/ml. The area under the receiver operating curve was 0.98 (0.93–1) for 18F-DOPA PET vs. 0.71 (0.43–0.95) for 68Ga-DOTANOC PET (p < 0.03). In patients subjected to surgery, localization of the focal lesion was correct in 91%, and 100%, by 18F-DOPA PET/CT and 68Ga-DOTANOC PET/CT, respectively.Conclusion18F-DOPA PET/CT was excellent in predicting focal CHI and superior compared to 68Ga-DOTANOC PET/CT. Further use of 68GA-DOTANOC PET/CT in predicting focal CHI is discouraged.
Highlights
Congenital hyperinsulinism (CHI) is a rare, heterogeneous disease characterized by inappropriate insulin secretion from pancreatic islet β-cells resulting in hypoglycaemia [1]
In patients subjected to surgery, localization of the focal lesion was correct in 91%, and 100%, by 18F-DOPA PET/CT and 68Ga-DOTANOC PET/CT, respectively
18F-DOPA PET/CT was excellent in predicting focal CHI and superior compared to 68Ga-DOTANOC PET/ CT
Summary
Congenital hyperinsulinism (CHI) is a rare, heterogeneous disease characterized by inappropriate insulin secretion from pancreatic islet β-cells resulting in hypoglycaemia [1]. The approximate incidence of persistent CHI is 1/40,000 live births in countries without founder mutations [2, 3]. The two major histological forms of CHI are the focal and the diffuse forms. Focal CHI occurs in 40–50% of cases [5] and is defined as a restricted pancreatic area with adenomatous β-cell hyperplasia, resulting in a lesion composed of confluent islets of Langerhans [6, 7]. Diffuse CHI involves the islets of Langerhans throughout the pancreas and is histologically characterized by hypertrophy of a few β-cell nuclei in most islets of Langerhans. Recessive inactivating mutations in ABCC8/KCNJ11 are the most common causes of medically unresponsive diffuse CHI [9], whereas dominantly inherited ABCC8/KCNJ11 mutations typically are medically responsive [1]
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