Controversy exists in the surgical treatment of patients with MEN 1 in relation to the timing and extent of interventions. Hyperparathyroidism in MEN 1 is caused by multiglandular disease and associated with high risk of supernumary glands. The findings on preoperative ultrasound and MIBI scans should not deter the surgeon from removing at least 3 parathyroid glands and the cervical thymus (1). Even subtotal parathyroidectomy (3½ glands) is associated with time dependant recurrence but lower risk of hypocalcaemia than total parathyroidectomy. The indications for surgical treatment of duodeno-pancreatic disease reflect the malignant potential of even small (<3 cm) pancreatic NET and syndromes of hormonal excess. High (>85%) biochemical cure rates can be achieved in gastrinoma patients, selective enucleation of pancreatic NETs is not indicated. Adrenal tumours > 10 mm in diameter, usually non-functioning, occur in at least 10% of individuals, MEN 1 is associated with an increased risk of adrenocortical cancer (2). MEN 1 related death occurs in approximately 70% of cases, increased risk is associated with thymic and duodenopancreatic tumours (3). In MEN 1 the heterogeneity of tumour penetrance and the risk of malignant transformation mandates lifelong follow up of gene carriers by an experienced multidisciplinary team. 1. Schreinemakers JM et al. The optimal surgical treatment for primary hyperparathyroidism in MEN1 patients: a systematic review. World J Surg 2011;35(9): 19932005. 2. Gatta B et al. Adrenal involvement in MEN1. Analysis of 715 cases from the Groupe d'etude des Tumeurs Endocrines database. Eur J Endocrinol 2011. 3. Goudet P. Risk factors and causes of death in MEN1 disease. A GTE (Groupe d'Etude des Tumeurs Endocrines) cohort study among 758 patients. World J Surg 2010;34(2): 249255.
Declaration of interest: There is no conflict of interest that could be perceived as prejudicing the impartiality of the research reported.
Funding: No specific grant from any funding agency in the public, commercial or not-for-profit sector.