Rapid response of Nelson's syndrome to pasireotide in radiotherapy-naive patient.

Xin He, Joanna L Spencer-Segal
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引用次数: 5

Abstract

Background: Nelson's syndrome is a well-described complication following bilateral adrenalectomy for management of Cushing's disease. There is no consensus on optimal management of Nelson's syndrome, characterized by the triad of pituitary corticotroph adenoma growth, elevated serum adrenocorticotropic hormone, and skin hyperpigmentation. Medical therapy with a variety of drug classes have been studied. One potentially promising drug already approved for Cushing's disease is pasireotide, a somatostatin analog with affinity for multiple somatostatin receptors, including subtype 5, the most highly expressed receptor on corticotroph tumors.

Case presentation: A 24-year-old female was diagnosed with Cushing's disease with initial ACTH levels around 700-800 pg/mL. She underwent transsphenoidal surgery without remission, followed by bilateral adrenalectomy. Over the subsequent 3 years, the patient developed skin hyperpigmentation, recurrent elevations of ACTH, and tumor recurrence requiring two additional transsphenoidal surgeries. After her third transsphenoidal resection, ACTH normalized, no residual tumor was seen on radiology, and the patient's skin hyperpigmentation improved. She then had an uncomplicated full-term pregnancy, during which ACTH levels remained within normal limits. One month after delivery, ACTH levels began rising to a peak at 5,935 pg/mL. Imaging revealed two new bilateral pituitary adenomas, measuring 14 mm on the left, and 7 mm on the right. She was then started on pasireotide. After two months of therapy, ACTH decreased to 609 pg/mL, and repeat pituitary MRI showed interval decrease in size of both pituitary adenomas to 13 mm on the left and 6 mm on the right.

Conclusion: We report the protracted course of a young female with several recurrences of Nelson's syndrome following bilateral adrenalectomy and multiple transsphenoidal surgeries, who ultimately responded to pasireotide. Unique features of her case not described previously are the response to pasireotide in a radiotherapy-naive patient, as well as the rapid radiologic response to therapy. Her history illustrates the unresolved challenges of Nelson's syndrome and the continued need for additional studies to identify optimal management.

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放射治疗初治患者纳尔逊综合征对帕西肽的快速反应。
背景:Nelson综合征是库欣病双侧肾上腺切除术后常见的并发症。对于以垂体促肾上腺皮质腺瘤生长、血清促肾上腺皮质激素升高和皮肤色素沉着为特征的纳尔逊综合征的最佳治疗方法尚无共识。已经研究了各种药物类别的医学治疗。已经批准用于库欣病的一种有潜力的药物是pasireotide,这是一种生长抑素类似物,对多种生长抑素受体具有亲和力,包括5亚型,这是促皮质性肿瘤中表达最高的受体。病例介绍:一名24岁女性被诊断为库欣病,初始ACTH水平约为700-800 pg/mL。她接受了经蝶窦手术,没有缓解,随后进行了双侧肾上腺切除术。在随后的3年中,患者出现皮肤色素沉着,ACTH复发性升高,肿瘤复发,需要另外两次经蝶窦手术。经第三次蝶窦切除术后,ACTH恢复正常,影像学未见肿瘤残留,皮肤色素沉着改善。然后她有一个简单的足月妊娠,在此期间ACTH水平保持在正常范围内。分娩一个月后,ACTH水平开始上升至5,935 pg/mL的峰值。影像学显示两个新的双侧垂体腺瘤,左侧14mm,右侧7mm。然后她开始服用帕西罗肽。治疗2个月后,ACTH降至609 pg/mL,复查垂体MRI示两垂体腺瘤大小间隔减小至左侧13 mm,右侧6 mm。结论:我们报告了一位年轻女性,在双侧肾上腺切除术和多次经蝶窦手术后多次复发纳尔逊综合征,最终对pasireotide有反应。该病例的独特特征是未接受放射治疗的患者对pasireotide的反应,以及对治疗的快速放射反应。她的病史说明了纳尔逊综合征尚未解决的挑战,以及继续需要进一步研究以确定最佳治疗方法。
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发文量
7
审稿时长
8 weeks
期刊介绍: Clinical Diabetes and Endocrinology is an open access journal publishing within the field of diabetes and endocrine disease. The journal aims to provide a widely available resource for people working within the field of diabetes and endocrinology, in order to improve the care of people affected by these conditions. The audience includes, but is not limited to, physicians, researchers, nurses, nutritionists, pharmacists, podiatrists, psychologists, epidemiologists, exercise physiologists and health care researchers. Research articles include patient-based research (clinical trials, clinical studies, and others), translational research (translation of basic science to clinical practice, translation of clinical practice to policy and others), as well as epidemiology and health care research. Clinical articles include case reports, case seminars, consensus statements, clinical practice guidelines and evidence-based medicine. Only articles considered to contribute new knowledge to the field will be considered for publication.
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