Characterization of Hyperinsulinism in Infancy Assessed with PET and 18F-Fluoro-L-DOPA

Characterization of Hyperinsulinism in Infancy Assessed with PET and ¹⁸F-Fluoro-L-DOPA

Maria-João Ribeiro, MD, PhD¹, Pascale De Lonlay, MD, PhD², Thierry Delzescaux, PhD¹, Nathalie Boddaert, MD³, Francis Jaubert, MD⁴, Sandrine Bourgeois¹, Frédéric Dollé, PhD¹, Claire Nihoul-Fékété, MD⁵, André Syrota, MD, PhD¹ and Francis Brunelle, MD³

¹ Service Hospitalier Frédéric Joliot, Département de Recherche Médicale, Direction des Sciences du Vivant, Commissariat à l’Energie Atomique, Orsay, France
² Département de Métabolisme et Pédiatrie, Hôpital Necker-Enfants Malades, Paris, France
³ Service de Radiologie Pédiatrique, Hôpital Necker-Enfants Malades, Paris, France
⁴ Laboratoire de Anatomopathologie, Hôpital Necker-Enfants Malades, Paris, France
⁵ Département de Chirurgie Infantile, Hôpital Necker-Enfants Malades, Paris, France

View larger version (35K):

[in a new window]

FIGURE 1. Patient with focal HI (patient 3). (A) Abnormal focal increased uptake of radiotracer is visualized in pancreas (P) on coronal and axial projections. Physiologic distribution of radiotracer with higher accumulation in kidneys (K) and urinary bladder (UB) and lower accumulation in liver (L) is also observed. (B) The 5 patients with focal HI forms were submitted to partial pancreatectomy. Abnormal hot spot corresponds to an important agglomeration of proinsulin, snaptophysin, chromogranin A, and AADC in adenoma (x100).

View larger version (83K):

[in a new window]

FIGURE 2. Patient with diffuse HI (patient 12). (A) Larger and diffuse uptake of radiotracer is observed over pancreatic area (P) (coronal and axial projections). Physiologic distribution of radiotracer with higher accumulation in kidneys (K) and urinary bladder (UB) and lower accumulation in liver (L) is also observed. (B) Images obtained for same patient after carbidopa administration: Note absence of ¹⁸F-fluoro-L-DOPA uptake by pancreas. (C) Corresponding immunohistochemical results (proinsulin, snaptophysin, chromogranin A, and AADC) obtained for same patient after subtotal pancreatectomy (x100).

View larger version (50K):

[in a new window]

FIGURE 3. Axial slices obtained from patient 8 with diffuse HI. (A) Seventy-two hours after drug withdrawal. (B) Under administration of octreotide and diazoxide. No differences in ¹⁸F-fluoro-L-DOPA pancreatic uptake were observed between the 2 PET studies. P = pancreas; L = liver; K = kidney.

View larger version (47K):

[in a new window]

FIGURE 4. MRI, enhanced MRI, PET, and resultant coregistration images obtained from patient 8 with diffuse HI: Coregistration images confirm diffuse uptake of ¹⁸F-fluoro-L-DOPA by pancreas (orange arrows = pancreas; yellow arrows = kidneys).

View larger version (27K):

[in a new window]

FIGURE 5. Time–activity curves (Bq/mL) in all measured organs between 50 and 80 min after intravenous injection of ¹⁸F-fluoro-L-DOPA. Curves were obtained for a patient with focal HI (A) and a patient with diffuse HI (B).