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This version published online on September 25, 2007
Journal of Clinical Endocrinology & Metabolism, doi:10.1210/jc.2007-1637
A more recent version of this article appeared on December 1, 2007
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Submitted on July 23, 2007
Accepted on September 17, 2007

Accuracy of [18F]-Fluorodopa PET for Diagnosing and Localizing Focal Congenital Hyperinsulinism

Olga T. Hardy, Miguel Hernandez-Pampaloni, Janet R. Saffer, Joshua S. Scheuermann, Linda M. Ernst, Richard Freifelder, Hongming Zhuang, Courtney MacMullen, Susan Becker, N. Scott Adzick, Chaitanya Divgi, Abass Alavi, and Charles A. Stanley*

Division of Endocrinology, Department of Radiology, Department of Pathology and Laboratory Medicine, and Department of Surgery, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania; and the Department of Radiology, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania

* To whom correspondence should be addressed. E-mail: stanleyc{at}email.chop.edu.

Objective: Focal lesions in infants with congenital hyperinsulinism represent areas of adenomatosis that express a paternally-derived KATP channel mutation due to embryonic loss of heterozygosity for the maternal 11p region. This study evaluated the accuracy of [18F]-DOPA PET scans in diagnosing focal versus diffuse disease and in identifying the location of focal lesions.

Design: Fifty infants with hyperinsulinism unresponsive to medical therapy were studied. Patients were injected intravenously with [18F]-DOPA and PET scans were obtained for 50–60 minutes. Images were co-registered with abdominal CT scans. PET scan interpretations were compared with histologic diagnoses.

Results: The diagnosis of focal or diffuse hyperinsulinism was correct in 44 of the 50 cases (88%). [18F]-DOPA PET identified focal areas of high uptake of radiopharmaceutical in 18 of 24 patients with focal disease. The locations of these lesions matched the areas of increased [18F]-DOPA uptake on the PET scans in all of the cases. PET scan correctly located 5 lesions that could not be visualized at surgery. The positive predictive value of [18F]-DOPA in diagnosing focal adenomatosis was 100% and the negative predictive value was 81%.

Conclusions: [18F]-DOPA PET scans correctly diagnosed 75% of focal cases and were 100% accurate in identifying the location of the lesion. These results suggest that [18F]-DOPA PET imaging provides a useful guide to surgical resection of focal adenomatosis and should be considered as a guide to surgery in all infants with congenital hyperinsulinism who have medically-uncontrollable disease.
Key words: congenital hyperinsulinism • [18F]-DOPA • hypoglycemia • nesidioblastosis • pancreatic islets • persistent hyperinsulinemic hypoglycemia of infancy • positron emission tomography




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 J. Clin. Endocrinol. Metab.Home page
W. Barthlen, O. Blankenstein, H. Mau, M. Koch, C. Hohne, W. Mohnike, T. Eberhard, F. Fuechtner, B. Lorenz-Depiereux, and K. Mohnike
Evaluation of [18F]Fluoro-L-DOPA Positron Emission Tomography-Computed Tomography for Surgery in Focal Congenital Hyperinsulinism
J. Clin. Endocrinol. Metab., March 1, 2008; 93(3): 869 - 875.
[Abstract] [Full Text] [PDF]


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