Total parathyroidectomy in a large cohort of cases with hyperparathyroidism associated with multiple endocrine neoplasia type 1: experience from a single academic center

Abstract

Most cases of sporadic primary hyperparathyroidism present disturbances in a single parathyroid gland and the surgery of choice is adenomectomy. Conversely, hyperparathyroidism associated with multiple endocrine neoplasia type 1 (hyperparathyroidism/multiple endocrine neoplasia type 1) is an asynchronic, asymmetrical multiglandular disease and it is surgically approached by either subtotal parathyroidectomy or total parathyroidectomy followed by parathyroid auto-implant to the forearm. In skilful hands, the efficacy of both approaches is similar and both should be complemented by prophylactic thymectomy.In a single academic center, 83 cases of hyperparathyroidism/multiple endocrine neoplasia type 1 were operated on from 1987 to 2010 and our first surgical choice was total parathyroidectomy followed by parathyroid auto-implant to the non-dominant forearm and, since 1997, associated transcervical thymectomy to prevent thymic carcinoid. Overall, 40% of patients were given calcium replacement (mean intake 1.6 g/day) during the first months after surgery, and this fell to 28% in patients with longer follow-up. These findings indicate that several months may be needed in order to achieve a proper secretion by the parathyroid auto-implant. Hyperparathyroidism recurrence was observed in up to 15% of cases several years after the initial surgery. Thus, long-term follow-up is recommended for such cases. We conclude that, despite a tendency to subtotal parathyroidectomy worldwide, total parathyroidectomy followed by parathyroid auto-implant is a valid surgical option to treat hyperparathyroidism/multiple endocrine neoplasia type 1. Larger comparative systematic studies are needed to define the best surgical approach to hyperparathyroidism/multiple endocrine neoplasia type 1.

Multiple endocrine neoplasia type 1 (MEN1)

“…3,52,53,58 As repeat parathyroid surgery is frequently technically more complicated, many surgeons elect to additionally transplant normal parathyroid tissue to a more accessible site on the forearm, although graft failure rates can be high. 52,59 The thymic remnant is also identified and excised during the operation, both to remove thymic ectopic parathyroid tissue and to prevent the development of MEN1-associated thymic neuroendocrine (NET) tumors (see "Other tumors"). As thymic NET have developed in a small number of cases post-surgery, some authors debate the effectiveness of this exercise.…”

“…23 Despite these measures, recurrent parathyroid disease is seen in up to 30% of patients following subtotal parathyroidectomy and patients should be warned that many will require further surgery within 10 years. 22,52,53,58,59 Surgery is indicated for symptomatic hyperparathyroidism, although medical treatment (bisphosphanates, calcimimetics) may be required if surgery is not feasible. 57 When an asymptomatic carrier of the MEN1 mutation is found during screening to have biochemical evidence of primary hyperparathyroidism, the timing of surgical intervention is controversial.…”

Successful parathyroid tissue autograft after 3 years of cryopreservation: a case report

“…Therefore, the acceptable surgical options for the treatment of HPT associated with familial or sporadic MEN1 are subtotal parathyroidectomy or total parathyroidectomy with an immediate autograft (20). Both techniques carry the risk of permanent hypoparathyroidism (12).…”

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

“…These tumors are usually the first clinical expression of MEN1 and are often diagnosed in the second decade of life (4,13). Hypercalcemia is usually mild, and severe hypercalcemia resulting in crisis is rare (5).…”

“…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).…”