Severe hypergastrinaemia with marked symptomatic rugal hypertrophy in a patient with MEN 1


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

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


A 54 year old female with a confirmed mutation in the MEN 1 gene, presented in 1990 with primary hyperparathyroidism and underwent a two-gland parathyroidectomy with a further completion parathyroidectomy in 1998 for recurrent hypercalcaemia. In 1990, she was also diagnosed with a gastrinoma (co-secreting insulin) treated with a proton-pump inhibitor.  In 2005, CTscan, revealed a 2.8cm lesion in the pancreatic head and two duodenal lesions (7.4mm and 7mm), consistent with neuroendocrine tumours (NET) with no change since 2003.  Octreotide scan confirmed increased uptake in the head of the pancreas consistent with NET. Visceral angiography with calcium stimulation and venous sampling in 2005 confirmed a rise of gastrin in superior mesenteric artery (SMA) and dorsal pancreatic artery (DPA) corresponding the lesions seen on CT scan (Basal gastrin  8000 to peak in SMA 23,700pmol/L and peak in DPA 28,700pmol/l) and a rise in insulin in the SMA and DPA (SMA:Basal insulin 9.0 to peak 46iu/L and DPA:basal of 66 to peak 82iu/L). In 2006, OGD and Colonoscopy were normal apart from moderate rugal hypertrophic gastritis with neuroendocrine cell hyperplasia on biopsy. Her current medication includes Omeprazole 40mg bd,  Thyroxine 50mcg od,  1-Alfacalcidol 0.75mcg od and Loperamide prn. On review this year, she was complaining of a significant increase in gastrointestinal symptoms with nausea, abdominal discomfort, intermittent diarrhoea and intermittent dysphagia. Abdominal MRI scan revealed that the enhancing lesion within the pancreatic head had markedly increased in size to 4.7 cm with no change in the two lesions in the duodenal wall. The gastric rugal hypertrophy had progressed significantly with the stomach lumen now almost totally obliterated. This lady is awaiting a Gallium-DOTOTATE PET scan and Barium meal.   In view of the marked progression of the pancreatic head lesion, rugal hypertrophy and symptomatology, in addition to a trial of octreotide therapy, we feel that this lady would benefit from surgical removal of pancreatic head and duodenal lesions as a debulking procedure and to reduce her hypergastrinaemia which should ameliorate her symptoms.