Bertagna Xavier, Guignat Laurence
Service des maladies endocriniennes et métaboliques, centre de référence des maladies rares de la surrénale, faculté de médecine Paris-Descartes, université Paris, hôpital Cochin, France.
Ann Endocrinol (Paris). 2012 Apr;73(2):107-10. doi: 10.1016/j.ando.2012.03.036. Epub 2012 Apr 24.
Transsphenoidal surgery, possibly through the endoscopic approach, remains the first line treatment. Opposing cortisol action with mifepristone proved efficacious in some individual cases but but with major monitoring difficulties. Combined treatment with three anticortisolic drugs (metyrapone, ketokonazole, O,p'DDD) is particularly attractive in severe cases. The Nelson's syndrome has been revisited, and the corticotroph tumor progression should rather be cautiously assessed after bilateral adrenalectomy. Two molecules potentially act directly to suppress the ACTH secretion by the corticotroph adenoma: agonists of the D2 Dopamine receptor and of the somatostatin receptor type 5. Their efficacy remains modest (20 to 30% of the patients actually normalize urinary cortisol). Pituitary radiotherapy can be efficiently performed by stereotaxic approach.
经蝶窦手术,可能通过内镜入路,仍然是一线治疗方法。在一些个别病例中,米非司酮拮抗皮质醇作用被证明是有效的,但存在重大监测困难。在严重病例中,三种抗皮质醇药物(甲吡酮、酮康唑、邻对滴滴滴)联合治疗特别有吸引力。尼尔森综合征已被重新审视,双侧肾上腺切除术后应谨慎评估促肾上腺皮质激素瘤的进展。有两种分子可能直接作用于抑制促肾上腺皮质激素腺瘤分泌促肾上腺皮质激素:D2多巴胺受体激动剂和5型生长抑素受体激动剂。它们的疗效仍然一般(实际只有20%至30%的患者尿皮质醇恢复正常)。垂体放疗可通过立体定向方法有效进行。