Espinosa-de-Los-Monteros Ana Laura, Sosa-Eroza Ernesto, Espinosa Etual, Mendoza Victoria, Arreola Rocio, Mercado Moises
Endocr Pract. 2017 Jul;23(7):759-767. doi: 10.4158/EP171756.OR. Epub 2017 Mar 23.
Treatment alternatives for persistent and recurrent Cushing disease (CD) include pituitary surgical re-intervention, radiation therapy (RT), pharmacotherapy, and bilateral adrenalectomy (BA). The decision of which of these alternatives is better suited for the individual patient rests on clinical judgment and the availability of resources. This retrospective cohort study was performed at a referral center to evaluate the long-term efficacy of different secondary interventions for persistent and recurrent CD.
We evaluated the hospital charts of 84 patients (77 female, median age 34 years, median follow up 6.3 years) with CD diagnosed, treated, and followed at our multidisciplinary clinic according to a pre-established protocol.
Of the 81 patients who were initially treated with transsphenoidal surgery (TSS), 61.7% had a long-lasting remission, 16% had persistent disease, and 22% achieved remission but relapsed during follow-up. The most frequently used secondary treatment was pituitary re-intervention, followed by ketoconazole, RT, and BA. Early remissions were observed in 66.6% of the re-operated and in 58.3% of the radiated patients; long-lasting remission was achieved in 33.3% and 41.6% of these patients, respectively. Nelson syndrome developed in 41.6% of the patients who underwent BA. Upon last follow-up, 88% of all the patients are in remission, and 9.5% are biochemically controlled with ketoconazole.
The efficacy of treatment alternatives for recurrent or persistent CD varies considerably among patients and multiple interventions are often required to achieve long-lasting remission.
ACTH = adrenocorticotrophic hormone; BA = bilateral adrenalectomy; CBG = cabergoline; CD = Cushing disease; CV = coefficient of variation; DXM = dexamethasone; IQR = interquartile range; RT = radiation therapy; SRS = stereotactic radiosurgery; TSS = transsphenoidal surgery; UFC = urinary free cortisol; ULN = upper limit of normal.
持续性和复发性库欣病（CD）的治疗选择包括垂体手术再次干预、放射治疗（RT）、药物治疗和双侧肾上腺切除术（BA）。这些治疗选择中哪种更适合个体患者的决定取决于临床判断和资源可用性。本回顾性队列研究在一家转诊中心进行，以评估不同二级干预措施对持续性和复发性CD的长期疗效。
我们根据预先制定的方案，评估了在我们多学科诊所诊断、治疗和随访的84例CD患者（77例女性，中位年龄34岁，中位随访6.3年）的医院病历。
在最初接受经蝶窦手术（TSS）治疗的81例患者中，61.7%获得持久缓解，16%疾病持续存在，22%缓解但在随访期间复发。最常用的二级治疗是垂体再次干预，其次是酮康唑、RT和BA。再次手术的患者中有66.6%和接受放射治疗的患者中有58.3%出现早期缓解；这些患者中分别有33.3%和41.6%获得持久缓解。接受BA的患者中有41.6%发生尼尔森综合征。在最后一次随访时，所有患者中有88%处于缓解状态，9.5%通过酮康唑实现生化控制。
复发性或持续性CD的治疗选择疗效在患者之间差异很大，通常需要多次干预才能实现持久缓解。
ACTH = 促肾上腺皮质激素；BA = 双侧肾上腺切除术；CBG = 卡麦角林；CD = 库欣病；CV = 变异系数；DXM = 地塞米松；IQR = 四分位数间距；RT = 放射治疗；SRS = 立体定向放射外科；TSS = 经蝶窦手术；UFC = 尿游离皮质醇；ULN = 正常上限
