Differences between sporadic and MEN related primary hyperparathyroidism; clinical expression, preoperative workup, operative strategy and follow-up

Orphanet Journal of Rare Diseases

Menno R Vriens²  Inne HM Borel Rinkes²  Gerlof D Valk¹  Anouk Scholten²  Bas A Twigt² 

[1] Department of Endocrinology, University Medical Center, Utrecht, the Netherlands;Department of Surgical Oncology and Endocrine Surgery, University Medical Center, Hp.nr. G04.228, Heidelberglaan 100, Utrecht, 3584CX, the Netherlands

关键词: Hypercalcemia;    Multiple endocrine neoplasia type 2;    Multiple endocrine neoplasia type 1;    PTH;    Familial hyperparathyroidism;    Parathyroid;   

Others  :  864062
DOI  :  10.1186/1750-1172-8-50

 received in 2012-10-03, accepted in 2013-02-04,  发布年份 2013

【 摘 要 】

Background

Primary hyperparathyroidism (PHPT) is most commonly sporadic (sPHPT). However, sometimes PHPT develops as part of multiple endocrine neoplasia (MEN) type 1 or 2A. In all, parathyroidectomy is the only curative treatment. Nevertheless, there are important differences in clinical expression and treatment.

Methods

We analyzed a consecutive cohort of patients treated for sporadic, MEN1-related, and MEN2A-related PHPT and compared them regarding clinical and biochemical parameters, differences in preoperative workup, operative strategies, findings, and outcome.

Results

A total of 467 patients with sPHPT, 52 with MEN1- and 16 with MEN2A-related PHPT were analyzed. Patients with sPHPT were older, more often female and had higher preoperative calcium and parathyroid hormone levels, when compared with MEN1 and MEN2A patients. Minimally invasive parathyroidectomy (MIP) was performed in 367 of 467 sPHPT patients (79%). One abnormal parathyroid was found in 426 patients (91%). Two or more in 35 patients (7%). In six patients (1%) no abnormal parathyroid gland was retrieved. Of 52 MEN1 patients, eight (15%) underwent a MIP and 44 patients (85%) underwent conventional neck exploration (CNE); with resection of fewer than 3½ enlarged glands in 21 patients (40%), subtotal parathyroidectomy (SPTX, 3-3½ glands) in seventeen (33%) and total parathyroidectomy with autotransplantation (TPTX) in six (12%). Eleven patients (21%) had persistent disease, 29 (56%) recurrent PHPT and nine (17%) permanent hypoparathyroidism, mostly after TPTX. Of 16 MEN2A patients, six (38%) underwent MIP, four (25%) CNE and six (38%) selective resection of the enlarged gland(s) during total thyroidectomy. Three patients (19%) suffered from persistent PHPT and two (13%) developed recurrent disease.

Conclusions

Sporadic PHPT, MEN1- and MEN2A-related PHPT are three distinct entities as is reflected preoperatively by differences in gender, age at diagnosis and calcium and PTH levels.

MEN2A patients are very similar to sPHPT with respect to operative approach and findings. MIP is the treatment of choice for both. MIP has low rates of persistent and recurrent PHPT and a low complication rate. The percentage of multiglandular disease and recurrences are significantly higher in MEN1 patients, demonstrating the need for a different approach. We advocate treating these patients with CNE and SPTX.

【 授权许可】

   
2013 Twigt et al.; licensee BioMed Central Ltd.

【 图 表 】

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