Purpose: Focal 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.
Methods: PET/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 (SUV) 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.
Results: Fifty-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 SUV ratio cut-off was 1.44 and the optimal 68Ga-DOTANOC PET SUV 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.
Conclusion: 18F-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.
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http://dx.doi.org/10.1007/s00259-017-3867-1 | DOI Listing |
Front Endocrinol (Lausanne)
December 2024
One Health Research Group, Universidad de las Americas, Quito, Ecuador.
Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors derived from chromaffin cells, with 80-85% originating in the adrenal medulla and 15-20% from extra-adrenal chromaffin tissues (paragangliomas). Approximately 30-40% of PPGLs have a hereditary component, making them one of the most genetically predisposed tumor types. Recent advances in genetic research have classified PPGLs into three molecular clusters: pseudohypoxia-related, kinase-signaling, and -signaling pathway variants.
View Article and Find Full Text PDFJ Endocrinol Invest
December 2024
Nuclear Medicine and Molecular Imaging, ICANS, Strasbourg University Hospitals, Strasbourg University, Strasbourg, France.
Purpose: To investigate the influence of germline succinate dehydrogenase (SDHx) pathogenic variants on 6-[F]-fluoro-3,4-dihydroxyphenylalanine (F-DOPA) Positron Emission Tomography (PET) radiomic signature of head and neck paragangliomas (HNPGLs).
Methods: Forty-seven patients (20 SDH pathogenic variants carriers) harboring 55 HNPGLs were retrospectively included. HNPGLs were delineated using Nestle adaptive threshold.
J Pediatr Endocrinol Metab
January 2025
Department of Infectious Diseases, University of Health Sciences, Bursa Yuksek İhtisas Training and Research Hospital, Bursa, Türkiye.
Objectives: Congenital syphilis can cause severe morbidities such as hydrops fetalis, preterm birth, low birth weight, hepatosplenomegaly, pneumonia, hypoglycemia, etc., and mortality. Despite hypoglycemia being reported in congenital syphilis and hyperinsulinism, only one case was described.
View Article and Find Full Text PDFQ J Nucl Med Mol Imaging
September 2024
Nuclear Medicine Unit, Department of Medical Sciences, AOU Città della Salute e della Scienza, Turin, Italy -
Radiol Med
September 2024
NESMOS, Department of Neuroradiology, S. Andrea Hospital, University Sapienza, Via di Grottarossa 1035/1039, 00189, Rome, Italy.
Objectives: To discriminate between post-treatment changes and tumor recurrence in patients affected by glioma undergoing surgery and chemoradiation with a new enhancing lesion is challenging. We aimed to evaluate the role of ASL, DSC, DCE perfusion MRI, and 18F-DOPA PET/CT in distinguishing tumor recurrence from post-treatment changes in patients with glioma.
Materials And Methods: We prospectively enrolled patients with treated glioma (surgery plus chemoradiation) and a new enhancing lesion doubtful for recurrence or post-treatment changes.
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