68Ga-DOTATATE PET- CT in the evaluation of Congenital Hyperinsulinism (#125)
Background: Congenital hyperinsulinism (CHI) is an uncommon disease characterized by severe and persistent hypoglycaemia, which can cause irreversible brain damage. Two main subtypes are described: diffuse and focal. Some genetic mutations may suggest focal or diffuse subtype of CHI, but genetic testing is currently not available worldwide. The incorporation of imaging studies in the evaluation of patients with CHI has changed the surgical management. 18F-DOPA PET/CT has proven highly accurate in distinguishing between focal and diffuse subtypes of CHI and is recommended as the first choice imaging modality. Unfortunately, this radiopharmaceutical is not available in most developing countries. 68Ga-DOTA peptide strongly binds to type II somatostatin receptors, which are highly concentrated in the neuroendocrine pancreatic islet cells and it can distinguish between focal or diffuse CHI.
Methodology: We present two cases of newborns with persistent hyperinsulinemic hypoglycaemia, with poor response to medical management. PET-CT was performed 35 minutes after intravenous administration of 1 mCi (37 MBq) of 68Ga-DOTATATE. The study was acquired in a Siemens Biograph 64 True Point camera, with a single PET bed position and low-dose CT for attenuation correction and anatomical correlation.
Results: 68Ga-DOTATATE PET-CT showed in one of the newborn a focal abnormal tracer uptake in the distal pancreatic body. The patient underwent partial pancreatectomy, guided by the image. In the other newborn, diffuse intense uptake was demonstrated, and after a year a good response to medical treatment. In both cases CHI was confirmed.
Conclusion: 68Ga-DOTA-peptide in CHI is a good alternative to 18F-DOPA able to distinguish focal from diffuse islet cell hyperplasia and allows a proper treatment and able to successfully guided surgery.