2026-04-13T02:20:38Zhttps://recercat.cat/oai/request

oai:recercat.cat:11351/51262025-10-24T10:40:57Zcom_2072_378070com_2072_378040col_2072_378092

00925njm 22002777a 4500 dc Lamas, Cristina author Navarro, Elena author Casteras Román, Anna author Portillo, Paloma author Calatayud, María author Mesa Manteca, Jordi author Alcázar, Victoria author 2020-07-31T12:20:43Z 2020-07-31T12:20:43Z 2019-10-01 Multiple endocrine neoplasia type 1; MEN1 gene; Primary hyperparathyroidism Neoplasia endocrina múltiple tipo 1; Gen MEN1; Hiperparatiroidismo primario Neoplàsia endocrina múltiple tipus 1; Gen MEN1; Hiperparatiroidisme primari Primary hyperparathyroidism is the most frequent manifestation of multiple endocrine neoplasia type 1 (MEN1) syndrome. Bone and renal complications are common. Surgery is the treatment of choice, but the best timing for surgery is controversial and predictors of persistence and recurrence are not well known. Our study describes the clinical characteristics and the surgical outcomes, after surgery and in the long term, of the patients with MEN1 and primary hyperparathyroidism included in the Spanish Registry of Multiple Endocrine Neoplasia, Pheochromocytomas and Paragangliomas (REGMEN). Eighty-nine patients (49 men and 40 women, 34.2 ± 13 years old) were included. Sixty-four out of the 89 underwent surgery: a total parathyroidectomy was done in 13 patients, a subtotal parathyroidectomy in 34 and a less than subtotal parathyroidectomy in 15. Remission rates were higher after a total or a subtotal parathyroidectomy than after a less than subtotal (3/4 and 20/22 vs 7/12, P < 0.05), without significant differences in permanent hypoparathyroidism (1/5, 9/23 and 0/11, N.S.). After a median follow-up of 111 months, 20 of the 41 operated patients with long-term follow-up had persistent or recurrent hyperparathyroidism. We did not find differences in disease-free survival rates between different techniques, patients with or without permanent hypoparathyroidism and patients with different mutated exons, but a second surgery was more frequent after a less than subtotal parathyroidectomy. The Spanish Registry of Multiple Endocrine Neoplasia, Pheochromocytmas and Paragangliomas is supported by IPSEN Pharmaceutical. This work did not receive any other specific grant from any funding agency in the public, commercial or not-for-profit sector. http://hdl.handle.net/11351/5126 Tiroide - Càncer Paratiroïdectomia Prognosi DISEASES::Neoplasms::Neoplasms by Site::Endocrine Gland Neoplasms::Multiple Endocrine Neoplasia::Multiple Endocrine Neoplasia Type 1 ANALYTICAL, DIAGNOSTIC AND THERAPEUTIC TECHNIQUES, AND EQUIPMENT::Surgical Procedures, Operative::Endocrine Surgical Procedures::Parathyroidectomy ANALYTICAL, DIAGNOSTIC AND THERAPEUTIC TECHNIQUES, AND EQUIPMENT::Diagnosis::Prognosis ENFERMEDADES::neoplasias::neoplasias::neoplasias primarias múltiples::neoplasia endocrina múltiple::neoplasias::neoplasia endocrina múltiple tipo 1 TÉCNICAS Y EQUIPOS ANALÍTICOS, DIAGNÓSTICOS Y TERAPÉUTICOS::intervenciones quirúrgicas::procedimientos quirúrgicos endocrinos::paratiroidectomía TÉCNICAS Y EQUIPOS ANALÍTICOS, DIAGNÓSTICOS Y TERAPÉUTICOS::Diagnóstico::Pronóstico MEN1-associated primary hyperparathyroidism in the spanish registry: clinical characterictics and surgical outcomes