Management of primary hyperparathyroidism caused by multiple gland disease

Abstract

Multiple gland parathyroid disease is the pathological finding in primary hyperparathyroidism (HPT) in about 10% to 20% of all patients and in approximately a third of all patients with persistent or recurrent disease. The variability of multiple gland disease spans from 2 adenomas to diffuse hyperplasia in patients with multiple endocrine neoplasia type 1. This variability calls into question the proposed common pathophysiologic background in all of these cases. As primary treatment of multiple gland primary HPT, subtotal parathyroidectomy and thymectomy or total parathyroidectomy and heterotopic autotransplantation including thymectomy can be equally advocated. Recurrent hyperparathyroidism frequently occurs in cases of diffuse parathyroid hyperplasia. This must be considered especially in patient follow up and before each surgical procedure. Thus, a defined but adaptable therapeutic regimen might prevent permanent hypoparathyroidism and persistent hyperparathyroidism.

A randomized, prospective trial of operative treatments for hyperparathyroidism in patients with multiple endocrine neoplasia type 1

“…Previous series of SP for HPT in MEN 1 report persistent or recurrent HPT in 0-65% of cases and permanent hypoparathyroidism in 8-35%. 5,7,8,[10][11][12][14][15][16][17][18][19][20][21]25 Series of total parathyroidectomy report recurrent HPT in 11-57% of cases and permanent hypoparathyroidism in #67%. 6,9,12,14,[18][19][20][21]23 This study represents the first prospective analysis of patients randomized to either SP or TP/AT with direct comparison of outcomes between the 2 groups.…”

“…Nevertheless, several retrospective series have compared the results of different operative procedures, collectively representing a large number of patients. [5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23] The interpretation of results from these comparisons is complicated by potential differences in patient selection, surgeon preferences or biases, variation in operative technique or extent of operative resection of the parathyroid glands, and lack of complete follow-up. Until the current study, a randomized, prospective evaluation of outcomes comparing the 2 commonly performed operations for HPT in MEN 1 has been lacking.…”

“…Despite the potential advantages and disadvantages of these 2 commonly practiced operations, previous retrospective studies have demonstrated similar overall rates of recurrent HPT and permanent postoperative hypoparathyroidism in patients undergoing the 2 operations. [5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23] Although one of these approaches is often preferred by individual expert endocrine surgery groups, improved outcomes or a clear advantage for either operation has not been established by previous studies. This randomized, prospective trial compared the results of TP/AT with SP for the treatment of patients with HPT and MEN 1.…”

The Outcome of Subtotal Parathyroidectomy for the Treatment of Hyperparathyroidism in Multiple Endocrine Neoplasia Type 1

“…The cure rate exceeds 90%. 1 Persistent and recurrent PHPT after operation usually occurs in the presence of multiple gland parathyroid disease, multiple adenomas, or diffuse hyperplasia of the parathyroid glands. 2,3 Multiple endocrine neoplasia type 1 (MEN 1) is an autosomal dominant condition typified by hyperplasia of the parathyroid glands, gastroenteropancreatic neuroendocrine neoplasia, and pituitary neoplasia.…”

“…9 However, other reports highlight a high rate of disease recurrence despite subtotal resection of the parathyroid glands. 1,10 Possible explanations for disparity between the findings of these studies are the inclusion of patients with sporadic hyperparathyroidism and an insufficient period of follow-up in those studies describing a good response to parathyroidectomy. 1,11 The cumulative recurrence rate for hyperparathyroidism in the re-port by O'Riordain et al 7 was 16.4% at 10 years; however, 41% of patients in this study did not have a history in the immediate family of MEN 1.…”