Diagnostic accuracy of [18F]-fluoro-l-dihydroxyphenylalanine positron emission tomography scan for persistent congenital hyperinsulinism in Japan

Abstract

We aimed to elucidate the accuracy and limitations of [(18)F]-fluoro-L-dihydroxyphenylalanine ([(18) F]DOPA) positron emission tomography (PET) for Japanese patients with congenital hyperinsulinism. Although [(18)F]DOPA PET is reported to be useful for precisely localizing the focal form of congenital hyperinsulinism, previous reports are mostly from European and North American centres. Seventeen Japanese infants with congenital hyperinsulinism. [(18)F]DOPA PET studies were carried out, and the results were assessed by simple inspection or by a quantitative measurement termed the 'Pancreas Percentage', which expresses the uptake of the head, body or tail of the pancreas as a percentage of the total maximum standardized uptake value of the whole pancreas. The results were compared with those of other studies, including genetic analysis and histology. By simple inspection, when a single focal uptake was obtained, the localization and histology were correct in all cases that underwent pancreatectomy. However, the overall results were consistent with the molecular diagnosis and histology in only 7/17 and 6/12 patients, respectively. The inaccuracy of PET studies by inspection was because of elevated background uptake that mimicked a diffuse or multifocal appearance. The accuracy improved substantially using the Pancreas Percentage; it was consistent with the molecular diagnosis and histology in 10/17 and 9/12 patients, respectively. In contrast to the results of previous reports, [(18)F]DOPA PET appears to be less efficient for diagnosing Japanese patients with congenital hyperinsulinism. However, the diagnostic accuracy is substantially improved when this technique is combined with the Pancreas Percentage.