Nelson syndrome: comprehensive review of pathophysiology, diagnosis, and management.
Nelson syndrome: comprehensive review of pathophysiology, diagnosis, and management.
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DOI：
10.3171/foc.2007.23.3.15
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发表时间：
2007-01-01
影响因子：
4.1
通讯作者：
Malek, Ali R
中科院分区：
文献类型：
--
作者：
Banasiak, Magdalena J;Malek, Ali R
纳尔逊综合征（NS）是一种罕见的临床表现，一个扩大的垂体腺瘤，可发生在双侧肾上腺切除治疗库欣病。其特征是促肾上腺皮质激素分泌过多以及皮肤和粘膜色素沉着过度。作者提出了一个全面审查的病理生理，诊断和管理NS。NS中的促肾上腺皮质激素腺瘤仍然是具有挑战性的肿瘤，可导致显著的发病率和死亡率。更好地了解NS的自然史，神经生理学和神经影像学的进步，以及手术干预和放射治疗经验的增加，扩大了治疗范围。目前可用的治疗方法包括手术、放射和药物治疗。虽然每种肿瘤类型的主要治疗可能会有所不同，但重要的是要考虑所有可用的选择，并选择最适合个体病例的治疗方案，特别是在病变对干预耐药的情况下。
Nelson syndrome (NS) is a rare clinical manifestation of an enlarging pituitary adenoma that can occur following bilateral adrenal gland removal performed for the treatment of Cushing disease. It is characterized by excess adreno-corticotropin secretion and hyperpigmentation of the skin and mucus membranes. The authors present a comprehensive review of the pathophysiology, diagnosis, and management of NS. Corticotroph adenomas in NS remain challenging tumors that can lead to significant rates of morbidity and mortality. A better understanding of the natural history of NS, advances in neurophysiology and neuroimaging, and growing experience with surgical intervention and radiation have expanded the repertoire of treatments. Currently available treatments include surgical, radiation, and medical therapy. Although the primary treatment for each tumor type may vary, it is important to consider all of the available options and select the one that is most appropriate for the individual case, particularly in cases of lesions resistant to intervention.