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Adrenal mass in a diabetic with hypergastrinaemia
  1. H M S Elashaa,
  2. D Devendraa,
  3. S Travisa,
  4. D Wilkinsb,
  5. P Newmanc,
  6. T J Wilkina
  1. aDerriford Hospital, Plymouth, UK: Department of Medicine, bDepartment of Surgery, cDepartment of Histopathology
  1. Dr H M S Elasha, Department of Medicine, West Cumberland Hospital, Whitehaven, Cumbria CA28 8TG, UK

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Answers on p 671.

A 50 year old man originally presented in 1980 at the age of 31 with diarrhoea, weight loss, and abdominal pain. He was initially treated with steroids for presumed inflammatory bowel disease. About a month later he presented with severe abdominal pain, and a laparotomy revealed multiple jejunal perforations and a possible mass in the head of the pancreas. He made a good recovery during which time a nasogastric tube was in place for two weeks. A markedly raised serum gastrin level confirmed the diagnosis of Zohlinger-Ellison syndrome. Other gut hormone levels were normal. A parathyroid adenoma was suspected on account of raised calcium and parathyroid hormone levels.

He underwent a parathyroidectomy, and since then his serum calcium has remained normal.

Biopsy of the nodule in the head of the pancreas confirmed a pancreatic islet cell tumour. He subsequently underwent total gastrectomy, distal oesophagectomy for a peptic oesophageal stricture, and pancreaticodoudenectomy in 1980.

His gastrin levels fell after surgery, but rose again sharply within the next two years.

He remained well until 1995, when he presented with weight loss, polyuria, and polydipsia.

Diabetes mellitus was diagnosed and he was started on insulin therapy. At this time a computed tomography scan of his abdomen revealed a 4 × 2 cm right adrenal mass and a 1.5 cm nodule in the tail of the pancreas. An octreotide labelled scan was normal, and the adrenal mass was thought to be an incidental mass. Subsequent computed tomography indicated that the adrenal mass was growing in size, and was removed surgically in 1999. Histology was diagnostic of primary adrenal carcinoma.

Questions

(1)
What other conditions besides Zohlinger-Ellison syndrome could cause a raised gastrin level?
(2)
Why does hypercalcaemia sometime present with abdominal pain?
(3)
How would you proceed to investigate whether the adrenal mass is functioning or just an incidental finding?
(4)
Why did this man develop diabetes mellitus?
(5)
Why is it important to screen his family?
(6)
Why did he develop a distal oesophageal stricture?

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