Cordeiro Diogo, Xu Zhiyuan, Li Chelsea E, Iorio-Morin Christian, Mathieu David, Sisterson Nathaniel D, Kano Hideyuki, Attuati Luca, Picozzi Piero, Sheehan Kimball A, Lee Cheng-Chia, Liscak Roman, Jezkova Jana, Lunsford L Dade, Sheehan Jason
1Department of Neurological Surgery, University of Virginia Health System, Charlottesville, Virginia.
2Department of Surgery, Division of Neurosurgery, Université de Sherbrooke, Centre de Recherche du CHUS, Sherbrooke, Quebec, Canada.
J Neurosurg. 2019 Jul 12;133(2):336-341. doi: 10.3171/2019.4.JNS19273. Print 2020 Aug 1.
Nelson's syndrome is a rare and challenging neuroendocrine disorder, and it is associated with elevated adrenocorticotrophic hormone (ACTH) level, skin hyperpigmentation, and pituitary adenoma growth. Management options including resection and medical therapy are traditional approaches. Ionizing radiation in the form of Gamma Knife radiosurgery (GKRS) is also being utilized to treat Nelson's syndrome. In the current study the authors sought to better define the therapeutic role of stereotactic radiosurgery (SRS) in Nelson's syndrome.
Study patients with Nelson's syndrome were treated with single-fraction GKRS (median margin dose of 25 Gy) at 6 different centers as part of an International Radiosurgery Research Foundation (IRRF) investigation. Data including neurological function, endocrine response, and radiological tumor response were collected and sent to the study-coordinating center for review. Fifty-one patients with median endocrine and radiological follow-ups of 91 and 80.5 months from GKRS, respectively, were analyzed for endocrine remission, tumor control, and neurological outcome. Statistical methods were used to identify prognostic factors for these endpoints.
At last follow-up, radiological tumor control was achieved in 92.15% of patients. Endocrine remission off medical management and reduction in pre-SRS ACTH level were achieved in 29.4% and 62.7% of patients, respectively. Improved remission rates were associated with a shorter time interval between resection and GKRS (p = 0.039). Hypopituitarism was seen in 21.6% and new visual deficits were demonstrated in 15.7% of patients.
GKRS affords a high rate of pituitary adenoma control and improvement in ACTH level for the majority of Nelson's syndrome patients. Hypopituitarism is the most common adverse effect from GKRS in Nelson's syndrome patients and warrants longitudinal follow-up for detection and endocrine replacement.
尼尔森综合征是一种罕见且具有挑战性的神经内分泌疾病，与促肾上腺皮质激素（ACTH）水平升高、皮肤色素沉着和垂体腺瘤生长有关。包括手术切除和药物治疗在内的管理方法是传统的治疗手段。伽玛刀放射外科手术（GKRS）形式的电离辐射也被用于治疗尼尔森综合征。在本研究中，作者试图更好地明确立体定向放射外科手术（SRS）在尼尔森综合征中的治疗作用。
作为国际放射外科研究基金会（IRRF）一项调查的一部分，6个不同中心对患有尼尔森综合征的研究患者进行了单次分割GKRS治疗（中位边缘剂量为25 Gy）。收集包括神经功能、内分泌反应和放射学肿瘤反应的数据，并发送至研究协调中心进行审查。对51例患者进行了分析，这些患者自GKRS治疗后的内分泌和放射学中位随访时间分别为91个月和80.5个月，分析内容包括内分泌缓解、肿瘤控制和神经学结果。使用统计方法确定这些终点的预后因素。
在最后一次随访时，92.15%的患者实现了放射学肿瘤控制。分别有29.4%和62.7%的患者在停止药物治疗后实现了内分泌缓解以及术前ACTH水平降低。缓解率的提高与切除手术和GKRS之间较短的时间间隔相关（p = 0.039）。21.6%的患者出现垂体功能减退，15.7%的患者出现新的视力缺陷。
对于大多数尼尔森综合征患者，GKRS能实现较高的垂体腺瘤控制率并改善ACTH水平。垂体功能减退是GKRS治疗尼尔森综合征患者最常见的不良反应，需要进行长期随访以检测和进行内分泌替代治疗。
