Hiperparatiroidismo primario asociado a neoplasia endocrina múltiple tipo 1 (MEN 1). Experiencia en 71 casos

Abstract

IntroductionPrimary hyperparathyroidism (pHPT) in MEN 1 is characterized by multiglandular disease and early involvement of parathyroid glands at different times. Persistence and recurrence range from 25%-35%.The purpose was: a) to describe the experience and the treatment of patients with pHPT in MEN 1; b) to analyze the variables related with clinical presentation and recurrence. MethodA total of 97 patients with MEN 1 were diagnosed in a tertiary hospital. A retrospective analysis was made in patients with pHPT (n=71). Study variables: age at diagnosis, mutation, clinical presentation, laboratory tests, surgical technique, and recurrence of HPT. ResultsMean age was 38 years, and 50 patients were asymptomatic. The surgical technique was: subtotal parathyroidectomy (n=55), resection of three glands (n=7), and resection of less glands (n=9). Transcervical thymectomy was performed in 53 patients. Mean follow-up was 102.9 months. There were 21 recurrences, There were correlations between age at diagnosis and serum calcium levels with the presence of symptoms (P<.0001). There were also correlations between recurrence and surgical technique (P<.03), non-association with thymectomy (P<.0001), and follow-up time (P<.03). ConclusionPerforming genetic and clinical screening allows us to make a diagnosis in the asymptomatic period and to provide early treatment for HPT in MEN 1. The recurrence rate is high, and follow-up time and the surgical technique used are risk factors for recurrence.