The added value of [18F]fluoro-L-DOPA PET in the diagnosis of hyperinsulinism of infancy: a retrospective study involving 49 children.
OBJECTIVE
Neuroendocrine diseases are a heterogeneous group of entities with the ability to take up amine precursors, such as L-DOPA, and convert them into biogenic amines, such as dopamine. Congenital hyperinsulinism of infancy (HI) is a neuroendocrine disease secondary to either focal adenomatous hyperplasia or a diffuse abnormal pancreatic insulin secretion. While focal hyperinsulinism may be reversed by selective surgical resection, diffuse forms require near-total pancreatectomy when resistant to medical treatment. Here, we report the diagnostic value of PET with [(18)F]fluoro-L-DOPA in distinguishing focal from diffuse HI.


METHODS
Forty-nine children were studied with [(18)F]fluoro-L-DOPA. A thoraco-abdominal scan was acquired 45-65 min after the injection of 4.2 +/- 1.0 MBq/kg of [(18)F]fluoro-L-DOPA. Additionally, 12 of the 49 children were submitted to pancreatic venous catheterisation for blood samples (PVS) and 31 were also investigated using MRI.


RESULTS
We identified abnormal focal pancreatic uptake of [(18)F]fluoro-L-DOPA in 15 children, whereas diffuse radiotracer uptake was observed in the pancreatic area in the other 34 patients. In children studied with both PET and PVS, the results were concordant in 11/12 cases. All patients with focal radiotracer uptake and nine of the patients with diffuse pancreatic radiotracer accumulation, unresponsive to medical treatment, were submitted to surgery. In 21 of these 24 patients, the histopathological results confirmed the PET findings. In focal forms, selective surgery was followed by clinical remission without carbohydrate intolerance.


CONCLUSIONS
These data demonstrate that PET with [(18)F]fluoro-L-DOPA is an accurate non-invasive technique allowing differential diagnosis between focal and diffuse forms of HI.
Biomedical Imaging Institute, Life Sciences Division, Commissariat à l'Energie Atomique, Frédéric Joliot Hospital, 4 place du Général Leclerc, Orsay, France. maria-joao.ribeiro@cea.fr
Maria-João
MJ
Ribeiro
Nathalie
N
Boddaert
Christine
C
Bellanné-Chantelot
Sandrine
S
Bourgeois
Vassili
V
Valayannopoulos
Thierry
T
Delzescaux
Francis
F
Jaubert
Claire
C
Nihoul-Fékété
Francis
F
Brunelle
Pascale
P
De Lonlay
D044903:Congenital Hyperinsulinism / Q000000981:diagnostic imaging*; D004295:Dihydroxyphenylalanine / Q000031:analogs & derivatives*; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D049268:Positron-Emission Tomography / Q000379:methods*; D019275:Radiopharmaceuticals; D015203:Reproducibility of Results; D012189:Retrospective Studies; D012680:Sensitivity and Specificity
D019275:Radiopharmaceuticals; C043437:fluorodopa F 18; D004295:Dihydroxyphenylalanine