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The added value of [18F]fluoro-L-DOPA PET in the diagnosis of hyperinsulinism of infancy: a retrospective study involving 49 children

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European Journal of Nuclear Medicine and Molecular Imaging Aims and scope Submit manuscript

Abstract

Purpose

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 [18F]fluoro-L-DOPA in distinguishing focal from diffuse HI.

Methods

Forty-nine children were studied with [18F]fluoro-L-DOPA. A thoraco-abdominal scan was acquired 45–65 min after the injection of 4.2 ± 1.0 MBq/kg of [18F]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 [18F]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.

Conclusion

These data demonstrate that PET with [18F]fluoro-L-DOPA is an accurate non-invasive technique allowing differential diagnosis between focal and diffuse forms of HI.

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Acknowledgements

We are greatly indebted to the chemical and nursing staff of Service Hospitalier Frédéric Joliot, Orsay, France. We are particularly grateful to Dr. Régine Trébossen and Dr. Claire Leroy for technical and advisory assistance, and to Dr. Christine Broissand, the Pharmacie Centrale des Hôpitaux de Paris and the GIS-Institut des Maladies Rares (Paris), for the temporary agreement for the use of carbidopa. We also thank the Fondation Lejeune.

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Authors and Affiliations

  1. Frédéric Joliot Hospital, Biomedical Imaging Institute, Life Sciences Division, CEA, 4, place du Général Leclerc, 91406, Orsay, France

    Maria-João Ribeiro, Sandrine Bourgeois & Thierry Delzescaux

  2. Department of Radiology, Necker-Enfants Malades Hospital, AP-HP, Paris, France

    Nathalie Boddaert & Francis Brunelle

  3. Department of Cytogenetics, Saint-Antoine Hospital, AP-HP, Paris, France

    Christine Bellanné-Chantelot

  4. Department of Pediatrics, Necker-Enfants Malades Hospital, AP-HP, Paris, France

    Vassili Valayannopoulos & Pascale De Lonlay

  5. Department of Pathology, Necker-Enfants Malades Hospital, AP-HP, Paris, France

    Francis Jaubert

  6. Department of Surgery, Necker-Enfants Malades Hospital, AP-HP, Paris, France

    Claire Nihoul-Fékété

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  1. Maria-João Ribeiro
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  2. Nathalie Boddaert
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  10. Pascale De Lonlay
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Correspondence to Maria-João Ribeiro.

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Ribeiro, MJ., Boddaert, N., Bellanné-Chantelot, C. et al. The added value of [18F]fluoro-L-DOPA PET in the diagnosis of hyperinsulinism of infancy: a retrospective study involving 49 children. Eur J Nucl Med Mol Imaging 34, 2120–2128 (2007). https://doi.org/10.1007/s00259-007-0498-y

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