Single Gland Excision for MEN1-Associated Primary Hyperparathyroidism

Jerena Manoharan; Max B. Albers; Carmen Bollmann; Elisabeth Maurer; Ioannis Mintziras; Sabine Wächter; Detlef K. Bartsch

Disclosures

Clin Endocrinol. 2020;91(1):63-70. 

In This Article

Abstract and Introduction

Abstract

Importance: Guidelines advocate subtotal parathyroidectomy (SPTX) or total parathyroidectomy with autotransplantation (TPTX) with bilateral cervical thymectomy for primary hyperparathyroidism (pHPT) associated with multiple endocrine neoplasia type 1 (MEN1). However, both procedures are associated with a significant risk of permanent hypoparathyroidism.

Objective: The aim of the current study was to compare long-term results of either single gland excision (SGE, 1–2 glands), SPTX and TPTX for the treatment of MEN1-associated pHPT.

Design and setting: Data of genetically confirmed MEN1 patients who underwent surgery for pHPT between 1987 and 2017 were retrieved from a prospective database and were retrospectively analysed.

Results: Eighty-nine MEN1 patients underwent either TPTX (n = 38, 42.7%), SPTX (n = 23, 25.8%) or SGE (n = 28, 31.5%). The rate of disease persistence after initial surgery was 2.6%, 0% and 14.2% in the TPTX, SPTX and SGE groups, respectively. After median follow-up of 112 (range 7–411) months, the rate of recurrent pHPT was significantly higher in the SGE group (n = 19, 21.3%) compared with the TPTX (n = 4, 4.4%, P = .001) and the SPTX (n = 9, 10.1%, P = .03) groups. Analysis of the recurrence-free time among the surgical groups revealed a significant difference (P = .036). The median time to recurrence was significantly shorter after SGE (101, range 3–301 months) than after SPTX (139, range 28–278 months, P = .018) and TPTX (204, range 75–396 months, P = .049). Twelve (32%) patients who underwent TPTX developed permanent hypoparathyroidism compared with only 4 (17%, P = .06) in the SPTX and 0 in the SGE group (P = .001).

Conclusion: Given the high rate of postoperative permanent hypoparathyroidism after TPTX and SPTX, SGE is a valid option for the treatment of MEN1-associated pHPT.

Introduction

Primary hyperparathyroidism (pHPT) is the most common and earliest manifestation in patients with multiple endocrine neoplasia type 1 (MEN1).[1–3] The penetrance of pHPT is over 95% by the age of 50.[2] In contrast to the sporadic form, MEN1-associated pHPT is characterized by an asymmetric multiglandular disease. MEN1-associated pHPT is not a life-threatening manifestation as pancreatic or thymic NEN, but due to its high penetrance and associated consequences of hypercalcemia, the management of this organ manifestation plays a pivotal role for MEN1 patients.[4–6]

Surgery is the preferred therapeutic approach in MEN1-associated pHPT. Compared to the sporadic form, surgical treatment in MEN1 patients is associated with a higher risk of recurrence and persistence of disease on one hand and permanent postoperative hypoparathyroidism on the other hand. The persistence or recurrence rate of MEN1-associated pHPT varies between 14% and 69% and the rate of permanent postoperative hypoparathyroidism with the need for life-long substitution therapy between 0% and 50% depending on the extent of surgery.[7,8] Thus, the optimal surgical approach is still matter of debate. Current clinical practice guidelines[1] recommend at least subtotal parathyroidectomy with bilateral cervical thymectomy (SPTX) or total parathyroidectomy with cervical thymectomy and autotransplantation of parathyroid tissue (TPTX). However, in the last years few authors have favoured a unilateral approach to avoid permanent postoperative hypoparathyroidism.[3,9–13] A unilateral approach or even focused single gland excision is considered justified, if preoperative diagnostic procedures (ultrasonography and/or 99mTc-MIBI scintigraphy) reveal unilateral or single enlarged parathyroid glands. In these cases, a targeted resection of one or two glands might be performed, if the intraoperative quick PTH measurement (IOPTH) reveals a drop of the intact serum parathyroid hormone level in the normal range after excision of hyperplastic gland(s). However, focused parathyroidectomy is controversially discussed, since the disease will definitively recur after some time and a reoperation is associated with a higher risk for complications, especially recurrent laryngeal nerve palsy. The aim of this study was to compare the long-term results of the different surgical procedures for the treatment of MEN1-associated pHPT and to evaluate which surgical procedure leads to durable remission and lowest risk of hypoparathyroidism.

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