Abstract
Background: The choice of surgical treatment for patients with Multiple Endocrine Neoplasia type 1 (MEN1)-related primary hyperparathyroidism (PHPT) remains controversial and it has not been specifically addressed in young patients.Methods: This is a retrospective case series study. The study includes the surgical data and the follow-up of 38 patients younger than 30 years of age, all diagnosed with MEN1, collected and followed-up between 1991 and 2017 at the Regional Referral Center for Inherited Endocrine Tumors of the Tuscany Region, and operated by parathyroidectomy. Genetic and/or clinical MEN1 diagnosis was made before surgery in all patients. Subtotal (9/38 patients) or total parathyroidectomy with auto-transplantation (28/38 patients) were performed in all patients but one, in whom a single mediastinal adenoma was excised from the aorto-pulmonary window. All patients but one, who was operated in 2017, had a post-operatory follow-up of at least 12 months.Results: Total parathyroidectomy (TPTX), with auto-transplantation, was the most frequently adopted operation both as primary (20/38 patients) and secondary (8/38 patients) surgery, followed by subtotal parathyroidectomy (SPTX; 9/38 patients) and limited parathyroidectomy (1/38 patient). At follow-up, lasting a mean of 11.8 ± 6.6 years (range 0–23 years), no persistent PHPT was observed. PHPT recurred in 4/28 TPTX (14%) and in 2/9 SPTX (22%). Permanent hypoparathyroidism showed no statistically significant difference between the procedures (2/9 in SPTX and 5/28 in TPTX).Conclusions: Data from this retrospective study showed the efficacy of TPTX for the treatment of MEN1-PHPT, also in adolescent and young patients, showing, in our series, no risk of PHPT permanence and a longer disease-free period and, subsequently, the possibility to postpone re-intervention with respect to both limited PTX and SPTX. The risk of permanent hypoparathyroidism in TPTX was comparable to STPX, and could be mitigated over the years.
Highlights
IntroductionPrimary hyperparathyroidism (PHPT) is the most common and, usually, the earliest endocrine manifestation in Multiple Endocrine Neoplasia type 1 (MEN1)
Primary hyperparathyroidism (PHPT) is the most common and, usually, the earliest endocrine manifestation in Multiple Endocrine Neoplasia type 1 (MEN1).Both timing and type of surgery for MEN1-primary hyperparathyroidism (PHPT) remain controversial and rarely investigated by clinical randomized trials
Parathyroid surgery grants the restoration of normal serum parathyroid hormone (PTH) and calcium level, but is beneficial for the control of gastrin oversecretion in MEN1 patients with a concomitant active gastrinoma [2]
Summary
Primary hyperparathyroidism (PHPT) is the most common and, usually, the earliest endocrine manifestation in Multiple Endocrine Neoplasia type 1 (MEN1). Both timing and type of surgery for MEN1-PHPT remain controversial and rarely investigated by clinical randomized trials. Post-surgical restoration of normal calcemia demonstrated to promote short-term partial recovery of trabecular bone mineral density at lumbar spine and femoral neck and to strongly reduce the manifestation of nephrolithiasis and related secondary kidney co-morbidities [3]. Transcervical thymectomy is usually performed, during both these procedures, to prevent thymic carcinoid and remove possible ectopic parathyroid glands [5]. The choice of surgical treatment for patients with Multiple Endocrine Neoplasia type 1 (MEN1)-related primary hyperparathyroidism (PHPT) remains controversial and it has not been addressed in young patients
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