Neuroendocrine Unit, Zero Emerson Place, Suite 112, Massachusetts General Hospital, Boston, MA 02114, USA.
Nat Rev Endocrinol. 2011 May;7(5):279-89. doi: 10.1038/nrendo.2011.12. Epub 2011 Feb 8.
Cushing disease is caused by a corticotroph tumor of the pituitary gland. Patients with Cushing disease are usually treated with transsphenoidal surgery, as this approach leads to remission in 70-90% of cases and is associated with low morbidity when performed by experienced pituitary gland surgeons. Nonetheless, among patients in postoperative remission, the risk of recurrence of Cushing disease could reach 20-25% at 10 years after surgery. Patients with persistent or recurrent Cushing disease might, therefore, benefit from a second pituitary operation (which leads to remission in 50-70% of cases), radiation therapy to the pituitary gland or bilateral adrenalectomy. Remission after radiation therapy occurs in ∼85% of patients with Cushing disease after a considerable latency period. Interim medical therapy is generally advisable after patients receive radiation therapy because of the long latency period. Bilateral adrenalectomy might be considered in patients who do not improve following transsphenoidal surgery, particularly patients who are very ill and require rapid control of hypercortisolism, or those wishing to avoid the risk of hypopituitarism associated with radiation therapy. Adrenalectomized patients require lifelong adrenal hormone replacement and are at risk of Nelson syndrome. The development of medical therapies with improved efficacy might influence the management of this challenging condition.
库欣病是由垂体的促肾上腺皮质激素细胞瘤引起的。库欣病患者通常采用经蝶窦手术治疗，因为这种方法可使 70-90%的病例得到缓解，并且由经验丰富的垂体外科医生进行时发病率较低。尽管如此，在术后缓解的患者中，手术后 10 年库欣病复发的风险可能达到 20-25%。因此，持续或复发的库欣病患者可能受益于第二次垂体手术（可使 50-70%的病例缓解）、垂体放疗或双侧肾上腺切除术。在相当长的潜伏期后，约 85%的库欣病患者在接受放疗后会缓解。由于潜伏期长，一般建议在患者接受放疗后进行临时药物治疗。对于经蝶窦手术后未改善的患者，特别是病情非常严重且需要快速控制皮质醇过多的患者，或希望避免与放疗相关的垂体功能减退风险的患者，可能会考虑双侧肾上腺切除术。接受肾上腺切除术的患者需要终身补充肾上腺激素，并且有发生纳尔逊综合征的风险。具有改善疗效的医学治疗方法的发展可能会影响这种具有挑战性疾病的治疗管理。
