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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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