Endocrine Abstracts

Multiple Endocrine Neoplasia Type 1(MEN1) syndrome is commonly associated with the three ’P’s-pituitary, parathyroid and pancreatic lesions. However, increasingly, thoracic neuroendocrine tumours (NETs) are being recognised with the syndrome. We present a case of MEN1 syndrome who primarily presented with a lung carcinoid tumour. A 31-year-old lady with without a significant past medical history presented with 2-month history of a persistent cough. Consequent chest x-ray and computed tomography (CT) scanning revealed a right middle lobe lung tumour, which was resected; histology revealed a typical carcinoid tumour (T2a N1 M0 R0). She did not have a family history of endocrine neoplasia. She subsequently underwent a whole body 68Ga-DOTATOC positive emission topography/CT scan, which showed three pancreatic NETs with magnetic resonance imaging detecting at least 2 of these sub centimetre intrapancreatic lesions (pancreatic head and uncinate process). She was commenced on monthly somatostatin analogue therapy. On biochemical screening, she had a raised serum adjusted calcium of 2.76 (2.2–2.60 mmol/l) and corresponding parathyroid hormone level of 8.2 (1.5–7.6 pmol/l) in keeping with primary hyperparathyroidism. Parathyroid imaging showed a focal 0.8 cm adenoma, and she is awaiting a subtotal parathyroidectomy. Her chromogranin A was 30 (0-91 mg/ml) and fasting gut hormones and basal pituitary profile were normal. Magnetic resonance imaging of her pituitary gland revealed a 0.4 cm cystic lesion. Genetic screening revealed a mutation in the MEN1 gene. This case highlights an unusual presentation of MEN1 syndrome with the initial symptoms being manifestations of a pulmonary NET. Previous studies have reported an estimated prevalence of thoracic NETs (thymic and pulmonary) of 2-8% with these being associated with premature mortality. It is therefore imperative that clinicians incorporate regular surveillance for thoracic NETs in their long-term management of MEN1 patients.