纳尔逊综合征：更新。

Nelson's Syndrome: An Update.

机构信息

Institute of Metabolism and Systems Research, College of Medical and Dental Sciences, University of Birmingham, IBR Tower, Level 2, Birmingham, B15 2TT, UK; Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham, B15 2TH, UK; Department of Endocrinology, Queen Elizabeth Hospital, University Hospitals Birmingham NHS Foundation Trust, Birmingham, B15 2TH, UK.

出版信息

Endocrinol Metab Clin North Am. 2020 Sep;49(3):413-432. doi: 10.1016/j.ecl.2020.05.004.

DOI:10.1016/j.ecl.2020.05.004

PMID:32741480

Abstract

Nelson's syndrome (NS) is a condition which may develop in patients with Cushing's disease after bilateral adrenalectomy. Although there is no formal consensus on what defines NS, corticotroph tumor growth and/or gradually increasing ACTH levels are important diagnostic elements. Pathogenesis is unclear and well-established predictive factors are lacking; high ACTH during the first year after bilateral adrenalectomy is the most consistently reported predictive parameter. Management is individualized and includes surgery, with or without radiotherapy, radiotherapy alone, and observation; medical treatments have shown inconsistent results. A subset of tumors demonstrates aggressive behavior with challenging management, malignant transformation and poor prognosis.

摘要

纳尔逊综合征（NS）是库欣病患者在双侧肾上腺切除术后可能发生的一种病症。虽然对于什么定义了 NS 还没有正式的共识，但促肾上腺皮质激素瘤的生长和/或逐渐增加的 ACTH 水平是重要的诊断要素。发病机制尚不清楚，也缺乏既定的预测因素；双侧肾上腺切除术后的头一年中高 ACTH 是最常报告的预测参数。治疗方法是个体化的，包括手术，联合或不联合放疗，单纯放疗和观察；药物治疗的结果不一致。有一部分肿瘤表现出侵袭性行为，具有挑战性的管理、恶性转化和预后不良。