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

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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., Matthae, R., Arya, V., Hauptmann, K., Rothe, K., Waechter, S., Singer, M., Mohnike, W., Eberhard, T., Raile, K., Lauffer, L.M., Iakoubov, R., 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
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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