Department of Internal Medicine, University Medical Center Utrecht, The Netherlands.
Ann Surg. 2012 Jun;255(6):1171-8. doi: 10.1097/SLA.0b013e31824c5145.
To identify the optimal surgical strategy for multiple endocrine neoplasia type 1 (MEN1)-related primary hyperparathyroidism (pHPT). To describe the course of postoperative hypoparathyroidism and to assess whether genotype is associated with persistent/recurrent pHPT.
Surgery is the preferred treatment in MEN1-related pHPT, but the surgical procedure of choice is still uncertain.
This retrospective cohort study was performed at the Departments of Endocrinology of the University Medical Centers of Utrecht and Nijmegen, the Netherlands. Patients were selected from the Dutch MEN1 database, including all patients 16 years or older treated for MEN1 from 1990 to 2009. Data were collected by medical record review.
Seventy-three patients underwent parathyroid surgery. Persistent/recurrent pHPT occurred in 53% after less than 3 parathyroids resected (<SPTX), 17% after subtotal resection (SPTX), and 19% after total resection with autotransplantation (TPTX). Persistent (≥6 months) postoperative hypoparathyroidism occurred in 24% after <SPTX, 39% after SPTX, and 66% after TPTX. Median duration of hypoparathyroidism was 1.5 years, in 65% successful cessation of vitamin D/calcium was possible, even after more than 10 years. After <SPTX, patients with nonsense or frameshift mutations in exons 2, 9, and 10 had a significantly lower risk of persistent/recurrent pHPT than patients with other mutations. After SPTX/TPTX persistence/recurrence did not differ with genotype. After SPTX/TPTX persistence/recurrence was more frequent (P = 0.07) in patients without bilateral transcervical thymectomy (TCT).
SPTX with bilateral TCT is the procedure of choice for MEN1-related pHPT. Genotype seems to affect the chance of recurrence. Postoperative hypoparathyroidism lasting 6 months or more should not be considered permanent in MEN1.
确定 1 型多发性内分泌肿瘤（MEN1）相关原发性甲状旁腺功能亢进症（pHPT）的最佳手术策略。描述术后甲状旁腺功能减退症的过程，并评估基因型是否与持续性/复发性 pHPT 相关。
手术是 MEN1 相关 pHPT 的首选治疗方法，但手术方式仍不确定。
本回顾性队列研究在荷兰乌得勒支和奈梅亨大学医学中心的内分泌科进行。从荷兰 MEN1 数据库中选择患者，包括 1990 年至 2009 年期间接受 MEN1 治疗的 16 岁及以上的所有患者。通过病历回顾收集数据。
73 例患者接受甲状旁腺手术。<3 个甲状旁腺切除（<SPTX）后，53%发生持续性/复发性 pHPT，次全切除（SPTX）后 17%，全切除伴自体移植（TPTX）后 19%。<SPTX 后 24%、SPTX 后 39%、TPTX 后 66%出现持续性（≥6 个月）术后甲状旁腺功能减退症。甲状旁腺功能减退症的中位持续时间为 1.5 年，65%的患者即使在 10 年以上仍可成功停止维生素 D/钙治疗。<SPTX 后，exon2、9 和 10 中存在无意义或移码突变的患者发生持续性/复发性 pHPT 的风险明显低于其他突变患者。SPTX/TPTX 后，基因型与持续性/复发性无关。SPTX/TPTX 后，无双侧经颈胸腺切除术（TCT）的患者（P = 0.07）更易发生持续性/复发性。
SPTX 联合双侧 TCT 是 MEN1 相关 pHPT 的首选手术方式。基因型似乎影响复发的几率。MEN1 中，持续 6 个月或更长时间的术后甲状旁腺功能减退症不应被视为永久性的。
