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Accuracy of [¹⁸F]Fluorodopa Positron Emission Tomography for Diagnosing and Localizing Focal Congenital Hyperinsulinism

Division of Endocrinology (O.T.H., C.M., S.B., C.A.S.), Departments of Radiology (H.Z.), Pathology and Laboratory Medicine (L.M.E.), and Surgery (N.S.A.), The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania 19104; and the Department of Radiology (M.H.-P., J.R.S., J.S.S., R.F., C.D., A.A.), University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104-4283

Address all correspondence and requests for reprints to: Charles A. Stanley, M.D., Division of Endocrinology, The Children’s Hospital of Philadelphia, 34th Street and Civic Center Boulevard, Philadelphia, Pennsylvania 19104. E-mail: stanleyc{at}email.chop.edu.

Objectives: Focal lesions in infants with congenital hyperinsulinism (HI) represent areas of adenomatosis that express a paternally derived ATP-sensitive potassium channel mutation due to embryonic loss of heterozygosity for the maternal 11p region. This study evaluated the accuracy of ¹⁸F-fluoro-L-dihydroxyphenylalanine ([¹⁸F]DOPA) positron emission tomography (PET) scans in diagnosing focal vs. diffuse disease and identifying the location of focal lesions.

Design: A total of 50 infants with HI unresponsive to medical therapy were studied. Patients were injected iv with [¹⁸F]DOPA, and PET scans were obtained for 50–60 min. Images were coregistered with abdominal computed tomography scans. PET scan interpretations were compared with histological diagnoses.

Results: The diagnosis of focal or diffuse HI 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 five 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 HI who have medically uncontrollable disease.

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