Machado Márcio Carlos, Fragoso Maria Candida Barisson Vilares, Moreira Ayrton Custódio, Boguszewski César Luiz, Vieira Neto Leonardo, Naves Luciana A, Vilar Lucio, Araújo Luiz Antônio de, Musolino Nina Rosa Castro, Miranda Paulo Augusto C, Czepielewski Mauro A, Gadelha Monica R, Bronstein Marcello Delano, Ribeiro-Oliveira Antônio
Unidade de Neuroendocrinologia, Serviço de Endocrinologia e Metabologia, Hospital das Clínicas da Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brasil.
Divisão de Endocrinologia e Metabologia, Faculdade de Medicina de Ribeirão Preto, Universidade de São Paulo, Ribeirão Preto, SP, Brasil.
Arch Endocrinol Metab. 2018 Feb;62(1):87-105. doi: 10.20945/2359-3997000000014.
The treatment objectives for a patient with Cushing's disease (CD) are remission of hypercortisolism, adequate management of co-morbidities, restoration of the hypothalamic-pituitary-adrenal axis, preservation of fertility and pituitary function, and improvement of visual defects in cases of macroadenomas with suprasellar extension. Transsphenoidal pituitary surgery is the main treatment option for the majority of cases, even in macroadenomas with low probability of remission. In cases of surgical failure, another subsequent pituitary surgery might be indicated in cases with persistent tumor imaging at post surgical magnetic resonance imaging (MRI) and/or pathology analysis of adrenocorticotropic hormone-positive (ACTH+) positive pituitary adenoma in the first procedure. Medical treatment, radiotherapy and adrenalectomy are the other options when transsphenoidal pituitary surgery fails. There are several options of medical treatment, although cabergoline and ketoconazole are the most commonly used alone or in combination. Novel treatments are also addressed in this review. Different therapeutic approaches are frequently needed on an individual basis, both before and, particularly, after surgery, and they should be individualized. The objective of the present review is to provide the necessary information to achieve a more effective treatment for CD. It is recommended that patients with CD be followed at tertiary care centers with experience in treating this condition.
库欣病（CD）患者的治疗目标包括高皮质醇血症缓解、合并症的妥善管理、下丘脑 - 垂体 - 肾上腺轴的恢复、生育能力和垂体功能的保留，以及对于伴有鞍上扩展的大腺瘤患者视觉缺陷的改善。经蝶窦垂体手术是大多数病例的主要治疗选择，即使是缓解可能性较低的大腺瘤。手术失败的情况下，若首次手术后磁共振成像（MRI）显示肿瘤持续存在和/或病理分析显示促肾上腺皮质激素阳性（ACTH+）垂体腺瘤，则可能需要再次进行垂体手术。经蝶窦垂体手术失败时，药物治疗、放射治疗和肾上腺切除术是其他选择。药物治疗有多种选择，尽管卡麦角林和酮康唑是最常用的单药或联合用药。本综述也讨论了新型治疗方法。无论是在手术前，尤其是手术后，通常都需要根据个体情况采用不同的治疗方法，且应个体化。本综述的目的是提供必要信息，以实现对CD更有效的治疗。建议CD患者在有治疗该疾病经验的三级医疗中心接受随访。
