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Occurrence of giant focal forms of congenital hyperinsulinism with incorrect visualization by (18)F DOPA-PET/CT scanning

Item Type:Article
Title:Occurrence of giant focal forms of congenital hyperinsulinism with incorrect visualization by (18)F DOPA-PET/CT scanning
Creators Name:Kuehnen, P. and Matthae, R. and Arya, V. and Hauptmann, K. and Rothe, K. and Waechter, S. and Singer, M. and Mohnike, W. and Eberhard, T. and Raile, K. and Lauffer, L.M. and Iakoubov, R. and Hussain, K. and Blankenstein, O.
Abstract:CONTEXT: Congenital hyperinsulinism (CHI) is a rare disease characterized by severe due to pathologically increased insulin secretion from the pancreatic beta-cells. When untreated, CHI might result in irreversible brain damage and death. Currently, two major subtypes of CHI are known: a focal form, associated with local distribution of affected beta-cells, and a non-focal form, affecting every single beta-cell. The identification of focal forms is important, as the patients can be cured by limited surgery. (18)F DOPA-PET/CT is an established non-invasive approach to differentiate focal from diffuse CHI. OBJECTIVE: The purpose of this study was to identify possible limitations of (18)F DOPA-PET/CT scan in patients with focal forms of CHI. DESIGN: A retrospective chart review of 32 patients (from 2008 through 2013) who underwent 18 F DOPA-PET/CT and partial pancreatectomy for focal CHI at the reference centers in Berlin, Germany and London, UK. RESULTS: In most cases (n=29, 90,7%), (18)F DOPA-PET/CT was sufficient to localize the complete focal lesion. However, in some patients (n=3, 9,3%), (18)F DOPA-PET/CT wrongly visualized only a small portion of the focal lesion. In this group of patients, a so-called "giant focus" was detected in histopathological analysis during the surgery. CONCLUSIONS: Our data shows that in most patients with focal CHI (18)F DOPA-PET/CT correctly predicts the size and anatomical localisation of the lesion. However, in those patients with a "giant focal" lesion (18)F DOPA-PET/CT is unreliable for correct identification of "giant focus" cases.
Keywords:Congenital Hyperinsulinism, Diagnostic Errors, Dihydroxyphenylalanine, Insulin-Secreting Cells, Multimodal Imaging, Pancreatectomy, Positron-Emission Tomography, Radiopharmaceuticals, Retrospective Studies, X-Ray Computed Tomography
Source:Clinical Endocrinology
ISSN:0300-0664
Publisher:Wiley-Blackwell (U.K.)
Volume:81
Number:6
Page Range:847-854
Date:December 2014
Official Publication:https://doi.org/10.1111/cen.12473
PubMed:View item in PubMed

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