Endocrine Abstracts (2002) 3 P127

Multiple endocrine neoplasia type 1 (MEN-1) with two different pancreatic tumours

S Kalavalapalli1, MT Barakat1, J Jackson2, JF Todd1, GR Williams1 & K Meeran1

1Imperial College School of Medicine Endocrine Unit, Hammersmith Hospital, London, UK; 2Department of Radiology, Hammersmith Hospital, London, UK;

A 29 year old woman was admitted to her local hospital with haematemesis, and found to be hypercalcaemic. A peptic ulcer was confirmed and she was commenced on omeprazole. There was biochemical evidence of primary hyperparathyroidism and a single-gland parathyroidectomy was performed. During her admission, a diagnosis of gastrinoma was suspected, but an attempt at measuring fasting gut hormone concentrations off omeprazole therapy resulted in gastro-intestinal perforation. She was referred for further investigation. The patient remained hypercalcaemic following transfer with a corrected calcium of 2.73mmol/l, and biochemical primary hyperparathyroidism. Her remaining 3 parathyroid glands were resected. A serum prolactin concentration was 811mIU/l (125-600) and pituitary MRI scanning revealed a 7mm pituitary tumour. During this time she reported symptoms of light-headedness and sweating at night relieved by eating. CT and endoscopic ultrasound of the pancreas revealed 2 lesions in the head of the pancreas and one in the tail. The lesions in the head of the pancreas were octreotide-avid, whereas the lesion in the tail was not. Visceral angiography with calcium stimulation was performed, and hepatic venous samples for gastrin and insulin were collected after selective cannulation of the pancreatic arteries. There were two angiographic blushes in the head of the pancreas in the territory supplied by the inferior pancreaticoduodenal and superior mesenteric arteries, and a significant rise in gastrin concentration was identified in these vessels. A third blush in the tail of the pancreas in the territory supplied by the splenic artery was associated with a marked rise in insulin secretion. A diagnosis of MEN-1 with primary hyperparathyroidism, microprolactinoma, two pancreatic head gastrinomas, and a pancreatic tail insulinoma was made. This case highlights the importance of considering the co-existence of more than one type of pancreatic tumour in MEN-1 and careful investigation should be performed in patients with complex symptoms.

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