Endocrine Abstracts

Introduction: Nesidioblastosis is a rare disease caused by hyperplasia of the pancreatic islets, resulting in hypoglycaemia due to increased insulin production. It is the main cause of hyperinsulin hypoglycaemia in children, although it accounts for only 0.5 to 5% of cases in adults. We report here the case of a patient with neidioblastosis successfully treated with lanreotide 120 mg LP, after persistent hypoglycaemia post-surgery.

Clinical case: A 62-year-old patient, not known to be diabetic, hypertensive on triple therapy, had been reporting deep hypoglycemia of 0.24 g/l for 2 years, with 3 to 4 episodes per week, mainly in the mornings. Abdominal MRI revealed a nodular formation at the corporo-caudal junction distorting the anterior contour of the pancreas with T2 hypersignal, T1 isosignal hypersignal measuring 21×18 mm. Biological tests revealed a C-peptide level of 2980 pmol/l (300-1400 pmol/l), and an insulin level of 676 pmol/l (18-173 pmol/l). The patient underwent two operations. Anatomopathological examination concluded to a nesidioblastosis. Immunohistochemistry was positive, with expression of anti-CK7 antibodies, anti-chromogranin antibodies and anti-synaptophysin antibodies. Given persistent hypoglycemia postoperatively, the patient was put on diazoxide 300 mg/d and hydrocortisone 60 mg/d without improvement, then Lanreotide 120 mg was added every 4 weeks. Daily monitoring did not reveal any hypoglycemia.

Discussion: The antisecretory effects of somatostatin on various hormones in endocrine organs can be regulated primarily by SSTR2 and SSTR5. They inhibit the counter-regulatory hormones glucagon and growth hormone (GH), which antagonize the effects of insulin. Lanreotide 120 mg LP is a long-acting somatostatin analogue. It is commonly used in the management of NETs. In the literature, cases of insulinoma successfully treated with lanreotide have been reported. However, no cases of nesidioblastosis have been reported.

Conclusions: Thus, in patients with neidioblastosis with hypoglycemia resistant to diazoxide treatment or in cases of poor tolerance of this treatment, the use of Lanreotide seems to be a therapeutic option to be considered.