Corticotroph tumor progression after adrenalectomy in Cushing's disease:: A reappraisal of Nelson's syndrome
Corticotroph tumor progression after adrenalectomy in Cushing's disease:: A reappraisal of Nelson's syndrome
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DOI：
10.1210/jc.2006-1328
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发表时间：
2007-01-01
影响因子：
5.8
通讯作者：
Bertagna, Xavier
中科院分区：
文献类型：
--
作者：
Assie, Guillaume;Bahurel, Helene;Bertagna, Xavier
背景：肾上腺切除术是治疗库欣病皮质醇增多症的根治性治疗方法。目的：本研究的目的是通过研究基于垂体磁共振成像扫描的皮质细胞肿瘤的进展和寻找预测因素来重新考虑纳尔逊综合征。设计：这是一项回溯性队列研究。背景：研究库欣病患者的完整医疗记录。患者：53例库欣病患者在1991-2002年间接受肾上腺切除术，没有接受过垂体放射治疗。测量：临床资料、垂体磁共振成像数据、垂体素放射治疗记录所有患者的血浆ACTH浓度和25例患者的脑垂体病理资料。用Kaplan-Meier法研究促肾上腺皮质细胞肿瘤无进展生存期，用Cox回归分析记录参数的影响。干预：无干预。结果：半数患者最终发生促肾上腺皮质细胞肿瘤进展，一般在肾上腺切除术后3年内。库欣病病程较短(调整后的风险比：0.884/年)，肾上腺切除术后一年的血浆促肾上腺皮质激素浓度较高[调整后的风险比每100pg/ml(22pmol/升)：1.069]是促肾上腺皮质细胞肿瘤进展的预测因素。在一个病例中，促肾上腺皮质细胞肿瘤进展合并一过性动眼神经麻痹。在随访期间，促肾上腺皮质激素的进展与相应的促肾上腺皮质激素浓度的升高相关(每100pg/ml促肾上腺皮质激素变异的优势比：1.055)。结论：库欣病肾上腺切除术后，不应再等待纳尔逊综合征的发生：现代影像可以及早发现和处理促肾上腺皮质激素的肿瘤进展。
Context: Adrenalectomy is a radical treatment for hypercortisolism in Cushing's disease. However, it may lead to Nelson's syndrome, originally defined by the association of a pituitary macroadenoma and high plasma ACTH concentrations, a much feared complication.Objective: The objective of the study was to reconsider Nelson's syndrome by investigating corticotroph tumor progression based on pituitary magnetic resonance imaging scan and search for predictive factors.Design: This was a retrospective cohort study.Setting: The complete medical records of Cushing's disease patients at Cochin Hospital were studied.Patients: Patients included 53 Cushing's disease patients treated by adrenalectomy between 1991 and 2002, without previous pituitary irradiation.Measurements: Clinical data, pituitary magnetic resonance imaging data, and plasma ACTH concentrations for all patients and pituitary gland pathology data for 25 patients were recorded. Corticotroph tumor progression-free survival was studied by Kaplan-Meier, and the influence of recorded parameters was studied by Cox regression.Intervention: There was no intervention.Results: Corticotroph tumor progression ultimately occurred in half the patients, generally within 3 yr after adrenalectomy. A shorter duration of Cushing's disease ( adjusted hazard ratio: 0.884/yr), and a high plasma ACTH concentration in the year after adrenalectomy [ adjusted hazard ratio per 100 pg/ml (22 pmol/liter): 1.069] were predictive of corticotroph tumor progression. In one case, corticotroph tumor progression was complicated by transitory oculomotor nerve palsy. During follow-up, corticotroph tumor progression was associated with the increase of corresponding ACTH concentrations (odds ratio per 100 pg/ml of ACTH variation: 1.055).Conclusion: After adrenalectomy in Cushing's disease, one should no longer wait for the occurrence of Nelson's syndrome: modern imaging allows early detection and management of corticotroph tumor progression.