[¹⁸F]-DOPA Positron Emission Tomography for Preoperative Localization in Congenital Hyperinsulinism

Abstract

In recent years, considerable progress has been made in the biochemical, morphological and molecular genetic differentiation of congenital hyperinsulinism (CHI). Fluorine-18 L-3,4-dihydroxyphenylalanine positron emission tomography (¹⁸F-DOPA-PET) has been introduced for differentiation between focal and diffuse CHI. The ability to take up L-DOPA and convert it into dopamine is correlated with the activity of the aromatic amino acid decarboxylase and increased in the hyperfunctional affected pancreatic area in comparison to normally functioning pancreas. The high sensitivity of this method allows the surgeon to perform a curative limited resection of a focus without the risk of long-term diabetes. The exact preoperative planning by ¹⁸F-DOPA-PET/CT computer tomography allows laparoscopic operation in selected cases with the focus in the tail and limits necessity to open the pancreatic duct in cases with focus in the head. Patients with persistent CHI should be managed within a strong network of diagnostic, treatment, and research institutions.

In vivo imaging of beta cells with radiotracers: state of the art, prospects and recommendations for development and use

“…Indeed, benign insulinomas are routinely localised against the pancreatic background using these agents [10]. In addition, it was shown that F-DOPA uptake could differentiate between the focal and diffuse forms of congenital hyperinsulinaemia, which in itself constitutes direct imaging of beta cell mass and dysfunction [11]. The full serotonergic metabolic pathway has recently been described in the pancreatic beta cell, as has been the expression of several serotonin receptors in beta, alpha and delta cells [12,13].…”

18F-FDOPA: A Multiple-Target Molecule

“…The focal form is caused by a paternally inherited ABCC8 or KCNJ11 gene mutation, whereas several other mutations are associated with the diffuse form, and in 50% of the cases the genetic basis is unknown (22). The introduction of 18 F-FDOPA PET and later PET/CT have made a tremendous change in the management of infants with the focal disease who can now be cured by molecular imaging-guided laparoscopic surgery (23). The sensitivity and specificity of 18 F-FDOPA PET for detecting a focus usually measuring between 2 and 10 mm is approximately 90% and 100%, respectively (21).…”

A Neonatal Case of Congenital Hyperinsulinism Due to Homozygous KCNJ11 Gene Mutation

“…Fluorine-18 L-dihidroksifenilalanin pozitron emisyon tomografi (18-F L-DOPA PET) taramasıyla, primer veya metastatik pankreas tümörü yanı sıra hiperfonksiyone pankreas hücreleri tanınabilir. Cerrahi girişim açısından yol gösterici nitelikte olan L-DOPA PET sayesinde lezyonun diffüz veya fokal olup olmama özelliği tanımlanabilir (15) . Olgumuzun yapılan batın MRG'sinde pankreas yapısı normal olarak değerlendirildi.…”