Removal of a pancreatic lesion for symptomatic relief in a patient with MEN 1

Simpson K, Troke R, Badman M, Goldstone AP, Todd JF.

Department of Endocrinology, Hammersmith and Charing Cross Hospitals, Imperial College Healthcare NHS Trust, Du Cane Road, London, W12 0HS


This 30 year old gentleman with MEN 1, confirmed on genetic testing, was  diagnosed after he developed primary hyperparathyroidism for which he had a two-gland parathyroidectomy  in 2008 (aged 29 years). He has remained normocalcaemic with normal PTH post operatively. Abdominal investigations revealed multiple insulinomas and gastrinomas within the pancreas. In 2008, abdominal CT and MRI scans revealed multiple lesions within the pancreas ( 2-3 in head (largest 14 mm), 1 in neck, 3 in tail (largest 3.3 cm)), consistent with neuroendocrine tumours (NETs).  Three were seen on angiography: one in head was supplied by anterosuperior pancreaticoduodenal artery; in neck by branch of dorsopancreatic artery (DPA) and one in tail by splenic Artery (SA); Calcium stimulation test and venous sampling showed increase insulin x12 from SA, x2 from IPDA, GDA, SMA and x1.6 from DPA but none from hepatic artery suggesting all three angiographic lesions were secreting insulin. Indium octreotide scan showed increased uptake probably in pancreatic head. He underwent enucleation of the NETs in pancreatic tail and head in February 2009.  The tail lesions confirmed low grade NET but no positive histology from excised lesion in head. However, post operatively he remained very symptomatic off a proton pump inhibitor (PPI) and also had continued episodes of hypoglycaemia. He was intolerant of Diazoxide. On post-operative 3-day fast, he became hypoglycaemic at 28h: glucose 2.0 mmol/L, insulin 6.8 mU/, C-Peptide at 523 pmol/L. Fasting gut hormones showed raised chromogranin A and B, gastrin 129pmol/L (NR <40), glucagon 70 pmol/L (NR <50).  Repeat abdominal CTscan showed at least 2 lesions in pancreatic head and persistent 3.5 cm lesion in tail of pancreas.  As the major source of insulin appeared to be secreted from the lesion in pancreatic tail which could be removed laparoscopically and may improve his QOL and as excision of the lesion in pancreatic head would require an open procedure, the patient underwent a laparoscopic distal pancreatectomy. Histology confirmed low grade NET (>50% cells stained for insulin). This has caused complete resolution of his hypoglycaemic symptoms with dramatic improvement to his QOL-he feels better that he has done for more than 10 years. However, he still requires a PPI without which he becomes very symptomatic.