Bariatric surgery in MEN1 and duodenal carcinoid - an interesting challenge
MSB Huda, DH Lewis, M Koteshwara, C Sinclair, D Kariyawasam A Patel, L Izatt, J Powrie, B McGowan
Guys and St. Thomas’ Hospitals, London.
A 27 year old lady presented with primary hyperparathyroidism and underwent a 3½ gland parathyroidectomy. Histology confirmed parathyroid hyperplasia. She had a positive family history and genetic testing confirmed MEN1.
She complained of weight gain, putting on 50kg over 5 years and with a present BMI of 56.7. Lifestyle changes and medical management were unsuccessful at achieving weight reduction. On examination, she had purple striae but no other features of Cushing syndrome. This was excluded by a low dose dexamethasone suppression test. Baseline pituitary function tests and a pituitary MRI were normal.
A fasting gut hormone profile showed a raised vasoactive intestinal peptide at 35 pmol/l (0-30 pmol/l) and a borderline chromogranin A at 61 pmol/l (0-59 pmol/l). Subsequent samples were within normal limits. An MRI of the abdomen did not reveal pancreatic abnormalities but an endoscopic ultrasound (EUS) showed a 2.5 mm lesion in the duodenum and a 3.5mm lesion in the uncinate of the pancreas. Histology of the duodenal biopsy demonstrated a well differentiated carcinoid tumour staining positive for chromogranin, synaptophysin and pan-cytokeratin (MNF116). Fine needle aspirate of the pancreatic lesion was inconclusive and we are monitoring this lesion with repeat EUS and MRI periodically.
Our patient was very keen to undergo bariatric surgery and was referred to the surgeons for consideration of this. Bariatric surgery poses a challenge in the context of a patient with MEN-1, carcinoid, and the possibility of future enteropancreatic and foregut pathology. Duodenal carcinoid has been associated with MEN-1 although uncommonly, and little is known about optimum treatment.
We are currently liaising with the bariatric surgeons to discuss management of the duodenal carcinoid, whether our patient should undergo bariatric surgery, and if so the optimal procedure. Our case illustrates the challenge of planning bariatric surgery in MEN1 syndrome.