Multiple endocrine neoplasia type 1 (MEN1) is defined clinically as the occurrence of two or more primary MEN1 tumour types, or in family members of a patient with a clinical diagnosis of MEN1, the occurrence of one of the MEN1-associated tumours.
A 70 years old man was incidentally diagnosed with hypercalcaemia in blood tests. Further investigations confirmed primary hyperparathyroidism. While awaiting parathyroid surgery, he was found to have a positive faecal occult blood test. Gastroscopy revealed duodenal ulcers, a biopsy from the ulcer diagnosed a low grade neuroendocrine tumour.
This is on the background of obesity, type 2 DM, gastro oesophageal reflux symptoms and atrial fibrillation.
Proton pump inhibitors (PPI) were ceased for blood tests for hormonal overproduction. He became symptomatic with episodes of flushing and dizzy spells and falls at home requiring hospital admission. He had severe orthostatic hypotension. While in hospital he developed upper GI bleeding and duodenal obstruction, which required HDU admission, but resolved with reinstitution of PPI.
Biochemical tests showed secretion of multiple hormones, including gastrin, glucagon, and pancreatic polypeptide. Dotatate PET scan showed intense uptake in second part of duodenum with multiple foci of tracer uptake within the pancreas. MRI abdomen- did not show any lesions other than the pancreatic and duodenal lesion.
Curative Whipple’s surgery was planned in view of the localised disease. At laparotomy, unexpectedly two liver lesions were found, which were confirmed to be metastatic neuroendocrine tumour, and surgery was abandoned.
He was commenced on Somatostatin analogues with improvement in symptoms, with ongoing follow-up.
Parathyroid glands were removed successfully. Genetic testing for MEN -1 is pending.
We briefly discuss the epidemiology and complications of MEN-1 to highlight how unusual the first presentation in this age group is.