Poster Presentation The Joint Annual Scientific Meetings of the Endocrine Society of Australia and the Society for Reproductive Biology 2017

Non-insulinoma pancreatogenous hypoglycaemia in adults (adult nesidioblastosis).  When distal pancreatectomy fails to effect improvement, consider using everolimus. (#264)

Yang Du 1 , Chris Karapetis 2 , Wilton J Braund 3
  1. Flinders Medical Centre, Bedford Park, SA, 5042
  2. Flinders Cancer Centre, Flinders Medical Centre, Bedford Park, SA, 5042
  3. Tennyson Centre, Kurralta Park, SA, Australia

Non-insulinoma pancreatogenous hypoglycaemia in adults (NIPHA, formerly 'adult nesidioblastosis') presents as hypoglycaemic episodes of varying severity.  Although regarded as extremely rare, NIPHA has been described more frequently in patients who have had gastric bypass surgery.

We describe a woman, first diagnosed at 44 years old, who experienced 'funny turns' that were diagnosed as hypoglycaemia by her GP, on the basis of a blood sample during a 'turn'.  The patient developed spontaneous hypoglycaemia within 4 hours of commencing a formal fasting investigation; as well as demonstrating hypoglycaemia in hospital upon waking.  Hypoglycaemia was accompanied by hyperinsulinaemia.  A qualitative screen did  not reveal the presence of sulphonylureas.

Imaging (CT scan, MRI of abdomen and a transgastric ultrasound of the pancreas) did not reveal a candidate lesion.  Transportal venous sampling showed multiple peaks of insulin at different parts of the pancreas.

At laparotomy, no candidate lesion could be identified; and a distal pancreatectomy was performed.  Histopathology revealed islet hyperplasia, islet proliferation and insulin-staining cells in pancreatic parenchyma and ducts.

Postoperatively, her hypoglycaemia worsened and appeared to be exacerbated by intermittent or continuously infused glucagon or somatostatin.  Diazoxide and calcium-channel blockers were ineffective.  Dexamethasone, 1.5mg to 2 mg per day, caused partial improvement.  Over the next few years, severe hypoglycaemic comas occurred - sometimes more than once a week - but the patient steadfastly declined a 95% pancreatectomy.

Since starting on everolimus 10mg daily, the patient has had partial relief from hypoglycaemia and complete relief from coma - except for its dramatic reappearance when everolimus was withdrawn because she had mouth ulcers. Comas have been eliminated since reintroduction of everolimus.  She has lost weight and has re-entered the workforce.

Although everolimus use has been reported in the management of insulinoma, we have not found reports of its use in NIPHA.

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