Torres-Ríos Jorge Alejandro, Romero-Luna Gerardo, Meraz Soto Juan Marcos, Zavala-Romero Lilian, Aguirre Maqueda Monica L, Rodríguez Camacho Alejandro, Moreno Jiménez Sergio
Neurosurgery and Radiosurgery, National Institute of Neurology and Neurosurgery, Mexico City, MEX.
Radiosurgery, National Institute of Neurology and Neurosurgery, Mexico City, MEX.
Cureus. 2023 May 16;15(5):e39114. doi: 10.7759/cureus.39114. eCollection 2023 May.
Nelson's syndrome (NS) is an uncommon disease occurring as a complication of bilateral adrenalectomy (BLA) in patients with persistent Cushing's disease (CD) due to an adrenocorticotropin-producing pituitary tumor. The first reports of this syndrome were done in the 50s, although its pathophysiology is still not understood. Every year, between 1.8 and 2.6 cases are thought to occur per million people. It is characterized by hyperpigmentation, elevated adrenocorticotropic hormone (ACTH) plasma levels, and typical signs and symptoms related to pituitary adenomas, such as visual deficits due to optic pathway compression or decreased hormone production from the adenohypophysis. NS represents a challenge due to the lack of accepted diagnostic criteria and the complexity of its treatment. Moreover, the development of stereotactic radiosurgery (SRS) in the last few years has become an essential but controversial strategy for this syndrome. This review presents a comprehensive overview of NS.
尼尔森综合征（NS）是一种罕见疾病，发生于因产生促肾上腺皮质激素的垂体肿瘤导致持续性库欣病（CD）的患者双侧肾上腺切除术后（BLA）。该综合征的首次报告于20世纪50年代完成，但其病理生理学仍未明确。据认为，每年每百万人中会出现1.8至2.6例。其特征为色素沉着过度、血浆促肾上腺皮质激素（ACTH）水平升高，以及与垂体腺瘤相关的典型体征和症状，如因视路受压导致的视力缺陷或腺垂体激素分泌减少。由于缺乏公认的诊断标准及其治疗的复杂性，NS是一项挑战。此外，近年来立体定向放射外科（SRS）的发展已成为该综合征的一项重要但存在争议的策略。本综述对NS进行了全面概述。
