Surgical approach in patients with hyperparathyroidism in multiple endocrine neoplasia type 1: total versus partial parathyroidectomy

Abstract

Usually, primary hyperparathyroidism is the first endocrinopathy to be diagnosed in patients with multiple endocrine neoplasia type 1, and is also the most common one. The timing of the surgery and strategy in multiple endocrine neoplasia type 1/hyperparathyroidism are still under debate. The aims of surgery are to: 1) correct hypercalcemia, thus preventing persistent or recurrent hyperparathyroidism; 2) avoid persistent hypoparathyroidism; and 3) facilitate the surgical treatment of possible recurrences. Currently, two types of surgical approach are indicated: 1) subtotal parathyroidectomy with removal of at least 3–3½ glands; and 2) total parathyroidectomy with grafting of autologous parathyroid tissue. Transcervical thymectomy must be performed with both of these procedures. Unsuccessful surgical treatment of hyperparathyroidism is more frequently observed in multiple endocrine neoplasia type 1 than in sporadic hyperparathyroidism. The recurrence rate is strongly influenced by: 1) the lack of a pre-operative multiple endocrine neoplasia type 1 diagnosis; 2) the surgeon's experience; 3) the timing of surgery; 4) the possibility of performing intra-operative confirmation (histologic examination, rapid parathyroid hormone assay) of the curative potential of the surgical procedure; and, 5) the surgical strategy. Persistent hyperparathyroidism seems to be more frequent after subtotal parathyroidectomy than after total parathyroidectomy with autologous graft of parathyroid tissue. Conversely, recurrent hyperparathyroidism has a similar frequency in the two surgical strategies. To plan further operations, it is very helpful to know all the available data about previous surgery and to undertake accurate identification of the site of recurrence.

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

“…Unsuspected diagnosis of MEN 1 at the time of initial operation, selective or less than SP, and surgeon inexperience are associated with greater rates of initial failure and subsequent recurrence. 22 The optimal timing of intervention is also important because of the inheritance of a genetic defect in every cell and the ''field effect'' of risk for neoplastic growth in all parathyroid tissue over time. The insidious development of initially mild HPT, with slow progression to more biochemically more evident disease over a decade or more, and the occurrence in otherwise young healthy patients are usually entirely asymptomatic at initial diagnosis, highlights the operative resection to perform.…”

“…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.…”

Metastatic Pancreatic Gastrinoma, Severe Primary Hyperparathyroidism, And Adrenal Cushing Syndrome In A Patient With Multiple Endocrine Neoplasia Type 1: An Unusual Presentation And Review Of The Literature

“…Intraoperative PTH measurement has become important to confirm the removal of all parathyroid tissue (14).…”

“…Hypercalcemia is usually mild, and severe hypercalcemia resulting in crisis is rare (5). Treatment is surgical (4) and most authors agree on the need to treat PHPT before other MEN1 endocrinopathies, with the exception of cases of insulinoma (14). Indications for parathyroidectomy in MEN1 should follow the same criteria used for sporadic asymptomatic PHPT (13).…”

“…Indications for parathyroidectomy in MEN1 should follow the same criteria used for sporadic asymptomatic PHPT (13). PHPT associated with MEN1 is surgically approached by either subtotal parathyroidectomy (removal of 3.5 glands, preserving at least 50 mg of parathyroid tissue) (7,11,13,14) or total parathyroidectomy followed by immediate parathyroid autograft (4,5,7,11,13).…”