Surgery for multiple endocrine neoplasia type 1-associated primary hyperparathyroidism

Abstract

Surgery in patients with multiple endocrine neoplasia type 1 (MEN1)-associated primary hyperparathyroidism (pHPT) is difficult as the condition it is caused by asymmetrical multiple gland hyperplasia. It is uncertain which operative procedure provides the best outcome with regard to long-term normocalcaemia. All patients who had surgery for genetically confirmed MEN1-associated pHPT between 1987 and 2009 were identified from a prospective database. Clinical data, operative procedures and outcome were analysed retrospectively. A total of 47 patients were identified. Twenty-three patients underwent total parathyroidectomy with thymectomy and autotransplantation (TPTX + AT), 11 patients subtotal parathyroidectomy (3-3.5 glands, SPTX) with thymectomy, and 13 patients selective gland excision (fewer than 3 glands, SGE). Rates of persistent disease, recurrent disease and permanent hypoparathyroidism after TPTX + AT were 4 per cent (1 patient), 4 per cent (1 patient) and 22 per cent (5 patients) respectively. Respective rates after SPTX were 0 per cent, 18 per cent (2 patients) and 45 per cent (5 patients), which were not statistically different from those following TPTX + AT. SGE resulted in persistent disease in 23 per cent (3 patients) and a significantly higher rate of recurrent disease (46 per cent, 6 patients; P = 0.004 versus TPTX, P = 0.210 versus SPTX), but permanent hypoparathyroidism did not occur. TPTX + AT and SPTX both seem adequate surgical procedures for the treatment of MEN1-associated pHPT and are associated with fewer recurrences than SGE.