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.

Metastatic disease rate was similar to that found in the literature. Perineural and angiolymphatic invasion and tumor thickness were associated with occult metastasis, but only angiolymphatic invasion showed to be an independent risk factor.

Background: The surgical treatment of primary hyperparathyroidism (HPT) in patients with multiple endocrine neoplasia type 1 (MEN1) has evolved due the concern of permanent hypoparathyroidism. As the diagnosis has increased, the extent of operation has decreased. Most MEN1 patients requiring parathyroidectomy are younger than 50 years and they pose a difficult balance to achieve between persistent HPT and life-long hypoparathyroidism. The aim of the present study is to review our experience with a large series of patients with MEN1-related HPT (HPT/MEN1) treated at a single institution in order to find clues to a better treatment decision in these younger cases.Method: Retrospective analysis of consecutive HPT/MEN1 cases treated at a single institution with different operations: total parathyroidectomy and immediate forearm autograft (TPTX-AG), subtotal (STPTX), unintentional less than subtotal (U-LSTPTX) and intentional less than subtotal parathyroidectomy (I-LSTPTX).Results: Considering 84 initial cases operated on since 2011 (TPTX-AG, 39; STPTX, 22, U-LSTPTX, 13, and I-LSTPTX, 10), the rates of hypoparathyroidism were 30.8% (U-LSTPTX), 28.2% (TPTX-AG), 13.6% (STPTX), and 0% (I-LSTPTX). Two-thirds of them (68%; 57/84) were young (<50 years) or asdolescents. MIBI scan was more sensitive to show parathyroid glands and bilateral disease. Considering the concordance of MIBI and ultrasound for the possibility of unilateral clearance, it would be suitable to 22.6% of the cases. Intra-operative parathormone showed a significant decay even after unilateral exploration, but longer follow up is necessary. Overall, there were seven (4%) adolescents in 161 cases treated from 1987 to 2018, three underwent TPTX-AG and four had U-LSTPTX. Five are euparathyroid, one had mild recurrence, and one required a reoperation after 8 years due to the residual gland.Conclusions: Young patients are the most frequent candidates to parathyroidectomy. Less extensive procedures may be planned only if carefully reviewed preoperative imaging studies suggest a localized disease. Patients and their relatives should be fully informed of the risks and benefits during consent process. Future research with larger cohorts and long-term results are necessary to clarify if less than I-LSPTX or unilateral clearance are really adequate in selected groups of patients with HPT/MEN1 presenting lower volume of disease detected by preoperative imaging studies.

The frequency of supernumerary parathyroid gland in the HPT1/MEN1 patients studied (12.2%) was significant. Surgeons should be aware of the need to search for supernumerary glands during neck exploration, besides the thymus. Imaging examinations were not useful in the pre-surgical location of these glands, and one case presented a recurrence of hyperparathyroidism.

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Background: Potential influences of the parathyroidectomy (PTx) on quality of life (QoL) in multiple endocrine neoplasia type 1-related primary hyperparathyroidism (HPT/MEN1) are unknown. Method: Short Form 36 Health Survey Questionnaire was prospectively applied in 30 HPT/MEN1 patients submitted to PTx (20, subtotal; 10, total with autograft), before, 6 and 12 months (mo.) after surgery. Parameters analyzed included QoL, age, HPT-related symptoms, general pain, comorbidities, biochemical/hormonal response, PTx type and parathyroid volume. Results: Asymptomatic patients were younger (30 vs 38 years; p=0.04) and presented higher QoL scores (physical component score, PCS, 92.5 vs 61.2 p=0.0051; mental component score, MCS, 82.0 vs 56.0, p=0.04) than symptomatic ones. In both groups, QoL remained stable one year after PTx, independently of number of comorbidities. Preoperative general pain was negatively correlated with PCS (r =-0.60, p=0.0004) and MCS (r=-0.57, p=0.0009). Also, moderate/intense pain was progressively (6/12mo.) more frequent in cases developing hypoparathyroidism. The PTx type and hypoparathyroidism did not affect the QoL at 12mo. although remnant parathyroid tissue volume did have a positive correlation (p=0.0490; r=0.3625) to PCS 12mo. after surgery. Patients with 1-2 comorbidities had as pre-PTx PCS (p=0.0015) as 12mo. post-PTx PCS (p=0.0031) and MCS (p=0.0365) better than patients with 3-4 comorbidities. Conclusion: A variable QoL profile was underscored in HPT/MEN1 reflecting multiple factors associated to this complex disorder as comorbidities, advanced age at PTx and presence of preoperative symptoms or of general pain perception. Our data encourage the early indication of PTx in HPT/MEN1 by providing known metabolic benefits to target-organs and avoiding potential negative impact on QoL.

Background: Surgery is the elective therapy for primary hyperparathyroidism (HPT) in patients with multiple endocrine neoplasia type 1 (MEN1). As MEN1 mutation carriers invariably will develop HPT up to 50 years, the annual periodic screening have allowed an early diagnosis of HPT in MEN1 syndrome, frequently before manifestation of clinical symptoms. Health-related quality of life (HR-QoL) both before after parathyroidectomy (PTx) has been poorly described. As the ideal timing to indicate PTx is yet controversial and undefined and, most recently, the value of less extensive surgeries has been considered for some groups, data on HR-QoL may be useful to clarify better these conflicting issues. Objectives: To investigate post-operative HR-QoL aspects of patients submitted to PTx for MEN1-related HPT (HPT/MEN1). Methods: Prospective analysis of HPT/MEN1 patients submitted to total PTx with immediate forearm autograft (TPTx+A) or subtotal parathyroidectomy (SPTx) in single institution. A psychologist applied Short Form 36 Health Survey Questionnaire immediately before and 6 months after surgery. Changes of both Physical Component Summary Score (PCS) and Mental Component Summary Score (MCS) were analyzed considering persistence of HPT, hypoparathyroidism and pancreatectomy after the PTx. Results: Of 19 patients (mean age 38.4±14.4, 7 men), 8 underwent TPTx+A and 11 had SPTx. Overall, median (interquartiles ranges) of pre-operative PCS and MCS were 73.8 (50.0-88.0) and 65.0 (44.5-83.2), respectively. At 6 months, median PCS was 71.2 (55.8-91.0) and median MCS was 80.2 (46.8-88.0). There were five (4, PTxT+A; 1, SPTx) patients with postoperative hypoparathyroidism, one with persistent HPT (SPTx) and 13 with normal parathyroid function (4, TPTx+A; 9, SPTx) six months after PTx. PCS worsened in four cases with hypoparathyroidism (80%) and in five euparathyroid (36%), but without statistical significance ( p , 0.14). MCS worsened in four cases with hypoparathyroidism (80%), but in only one euparathyroid (7%) ( p , 0.0061). The worst decrease of both PCS and MCS occurred in two patients submitted to pancreatectomy during the follow-up after parathyroidectomy (PCS decreased 79% in one case and 53% in the other; MCS decreased 75% and 37%, respectively). Conclusion: Hypoparathyroidism worsens the HR-QoL after PTx in HPT/MEN1, particularly in patients undergoing subsequent pancreatectomy.