P005 and OC2

Glucagonoma in MEN1: a case for total pancreatectomy?

S Sivappriyan, T Kurzawinski and J Ahlquist

Southend University Hospital, and University College Hospital.

Abstract:

The glucagonoma syndrome is caused by a glucagon secreting tumour arising in the alpha cells of the pancreatic islets; the clinical picture includes diabetes mellitus, migratory erythema and venous thrombosis. We describe here a patient with glucagonoma syndrome who presents a particular management challenge. A 59 year old man was referred with a new diagnosis of diabetes. He was known to have MEN1. Asymptomatic primary hyperparathyroidism had been diagnosed incidentally at the age of 31 years, and he had undergone 3 gland parathyroidectomy. He developed renal stones two years later; two further parathyroid explorations were initially successful but he subsequently developed recurrent mild asymptomatic hypercalcaemia (calcium 2.8 mmol/l). At 46 he had suffered marked peptic ulceration and spontaneous hypoglycaemia, and was found to have multifocal pancreatic tumours secreting both insulin and gastrin; following distal pancreatectomy and enucleation of two tumours in the head of pancreas he had remained well for 10 years. There was no history of pituitary disease. In the year prior to the recent diagnosis of diabetes he had suffered from recurrent DVT and a rash which was initially attributed to an allergy to anticoagulants. Recently his appetite has been poor and he has lost weight. On examination there was a scaly erosive troublesome rash affecting abdomen, genitals and limbs. Serum glucagon was elevated at 375 and >500 (normal <50) pmol/l; pancreatic polypeptide and chromogranin were also slightly elevated. CT showed no clear evidence of a tumour in the residual pancreas. Radionuclide imaging with a GLP1 receptor ligand (exendin CT) was negative but an octreotide scan indicated tumour in the head of pancreas and an adjacent node. Surgery will require completion pancreatectomy and lymph node clearance. The panel is invited to consider and discuss the relative benefits and hazards to the patient of this approach.