Endocrine Abstracts

Introduction: Insulinomas are the most common, functioning, pancreatic neuro-endocrine tumours. The minority of patients <10% who present with metastatic disease have a median survival of <2 years.

We present a case of a gentleman with a 30 years history of Multiple Endocrine neoplasia type 1 (MEN1), which highlights the various modalities of treatment and the challenges from his progressive disease and marked symptomatic hypoglycaemia.

Case History: Mr R W, a 75-year-old patient was first diagnosed with primary hyperparathyroidism in 1972. He underwent parathyroidectomy and had a recurrence in 2002 requiring repeat surgery. Genetic testing confirmed MEN-1. His son also was found to have MEN-1. In 2005, he was diagnosed with Insulinoma with symptomatic hypoglycaemic episodes. At the time of diagnosis, he had metastatic lesions in the liver and spleen in addition to the pancreatic lesion.

In 2006 he underwent surgery including distal pancreatectomy, splenectomy and resection of liver lesions. In addition radiofrequency ablation of liver lesions was also performed. Due to his clinical and radiologically progressive disease, he underwent three further radiofrequency ablations in 2007. An Octreotide trial in 2008 failed to give symptomatic relief. Progressive disease necessitated another surgery in 2009, followed by further radiofrequency ablation.

Diazoxide provided limited symptomatic relief but with significant side effects.

In view of his on-going hypoglycaemic episodes and inability to tolerate higher doses of diazoxide, he was initiated on Everolimus in June 2012. We show, with continuous glucose monitoring, that the frequency of his hypoglycaemic episodes has reduced significantly. It is too early to comment on disease resolution.

Conclusions: Everolimus is a highly promising treatment for metastatic insulinoma that combines direct anti-tumour effects with hyperglycaemic effects that serve to relieve the morbidity from hypoglycaemia.