A complex case of MEN1

Dukhabandhu Naik; D M Mahesh; H S Asha; Deepak T Abraham; Nylla Shanthly; Anuradha Chandramohan; Deepak Burad; Banumathi Ramakrishna; M J Paul; Nitin Kapoor; Thomas Paul; Ari G Chacko; Nihal Thomas

doi:10.1530/endoabs.38.P101

Objective: We report the rare case of a 45-year-old male with multiple endocrine neoplasm 1 (MEN1) with simultaneous occurrence characteristic tumours leading to a delicately balanced metabolic homeostasis.

Methods: Clinical, biochemical, and radiological data of this patient diagnosed to have MEN1 were analysed and the challenges in the management of these neuroendocrine tumours are discussed.

Case illustration: A 45-year-old gentleman presented with hip pain, acral enlargement, erectile dysfunction, and early morning hypoglycaemic symptoms. He was diagnosed to have MEN1 with primary hyperparathyroidism, acromegaly due to GH and prolactin co-secreting Hardy’s grade E pituitary macroadenoma, pancreatic neuro-endocrine tumours (PNETs) with biochemical evidence of both hyperinsulinaemic hypoglycaemia and hypergastrinemia. He also had collagenomas, lipomas, and non-functioning thymic and bilateral adrenal adenomas. He had a delicate metabolic homeostasis due to GH and insulin counterbalancing the glucose and; parathormone and GH counterbalancing serum phosphate. In view of limited mobility due to hip pain, proximal myopathy, and secondary osteoporosis he first underwent three and 1/2 gland parathyroidectomy in 2012. Acromegaly, hyperprolactinemia, and the PNETs were initially medically managed with octreotide LAR and cabergoline. Subsequently, in 2013 he underwent subtotal excision of the pituitary macrodadenoma. After pituitary surgery he had worsening of hypoglycemic episodes despite diazoxide therapy. Hence he underwent distal pancreatectomy with enucleation of PNETs in the head and uncinate process of pancreas, excision of duodenal (D2) submucosal tumours and omental lymphnode dissection. Histopathology confirmed multiple NETs in pancreas, duodenum and lymphnodes, with tumour immunopositivity for pancytokeratin, synaptophysin, and chromogranin (MIB1 index 2–3%). Following the resection of NETs, his GH levels declined; however he was continued on cabergoline for persistent hyperprolactinaemia.

Conclusion: The present complex case of MEN1 highlights the challenges in the management; necessitating a multidisciplinary team approach in view of the delicately balanced homeostasis, multicentric tumours, and risk of recurrence.

Endocrine Abstracts

P101