Accuracy of [18F]fluorodopa positron emission tomography for diagnosing and localizing focal congenital hyperinsulinism
Department of Pediatrics
Biopsy; Dihydroxyphenylalanine; Humans; Hyperinsulinism; Image Interpretation, Computer-Assisted; Infant; Infant, Newborn; Islets of Langerhans; Kidney; Multiple Endocrine Neoplasia; Pancreatectomy; Pancreatic Neoplasms; Positron-Emission Tomography; Radiopharmaceuticals; Sample Size
Endocrinology, Diabetes, and Metabolism | Pediatrics
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.
DOI of Published Version
J Clin Endocrinol Metab. 2007 Dec;92(12):4706-11. Epub 2007 Sep 25. Link to article on publisher's site
The Journal of clinical endocrinology and metabolism
Hardy OT, Hernandez-Pampaloni M, Saffer JR, Scheuermann JS, Ernst LM, Freifelder R, Zhuang H, MacMullen C, Becker S, Adzick NS, Divgi C, Alavi A, Stanley CA. (2007). Accuracy of [18F]fluorodopa positron emission tomography for diagnosing and localizing focal congenital hyperinsulinism. Endocrinology/Diabetes. https://doi.org/10.1210/jc.2007-1637. Retrieved from https://escholarship.umassmed.edu/peds_endocrinology/10