The Optimal Surgical Treatment for Primary Hyperparathyroidism in MEN1 Patients: A Systematic Review

The optimal surgical approach for patients with primary hyperparathyroidism (pHPT) and multiple endocrine neoplasia 1 (MEN1) is controversial. We sought to determine the optimal type of surgery for pHPT in MEN1. We collected data on clinical presentation, surgery, and follow-up for MEN1 patients with pHPT at the University Medical Center Utrecht and affiliated hospitals between 1967 and 2008. Furthermore, we performed a systematic review of the literature and meta-analysis. Surgical procedures were classified into less than subtotal (< SPTX) versus subtotal (SPTX) and total parathyroidectomy (TPTX). Fifty-two patients underwent primary surgery for pHPT, of which 29 had < SPTX, 17 SPTX, and 6 TPTX. Recurrent pHPT was most frequent after SPTX (65%) followed by < SPTX (59%). Persistent disease was most frequent after < SPTX (31%). Time to recurrence was 61 months longer after SPTX than after < SPTX. Although recurrent pHPT was not seen after TPTX, permanent hypoparathyroidism developed in 67% of these patients. The meta-analysis showed that after SPTX and TPTX, patients had the lowest risk of persistent and recurrent pHPT. TPTX had the highest risk of permanent hypoparathyroidism. Large noncomparative studies showed a low recurrence rate after SPTX and TPTX. We believe that SPTX is the best surgical therapy for pHPT in MEN1. MEN1 patients with pHPT should not be treated with < SPTX because of the unacceptable high rate of recurrent and persistent pHPT. Additionally, a thymectomy should routinely be performed in these patients.