Rapid response of Nelson's syndrome to pasireotide in radiotherapy-naive patient.
Rapid response of Nelson's syndrome to pasireotide in radiotherapy-naive patient.
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DOI：
10.1186/s40842-020-00110-7
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发表时间：
2020-11-07
影响因子：
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通讯作者：
Spencer-Segal JL
中科院分区：
文献类型：
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作者：
He X;Spencer-Segal JL
纳尔逊综合征是一个众所周知的并发症后，双侧肾上腺切除术的管理库欣病。对于纳尔逊综合征的最佳治疗尚无共识，其特征为垂体促肾上腺皮质激素腺瘤生长、血清促肾上腺皮质激素升高和皮肤色素沉着。已经研究了各种药物类别的药物治疗。一种已经批准用于库欣病的潜在有希望的药物是帕瑞肽，一种生长抑素类似物，对多种生长抑素受体具有亲和力，包括亚型5，这是促肾上腺皮质激素肿瘤中表达最高的受体。一名24岁女性被诊断患有库欣病，初始ACTH水平约为700-800 pg/mL。她接受了经蝶手术，没有缓解，随后双侧肾上腺切除术。在随后的3年中，患者出现皮肤色素沉着过度、ACTH复发性升高和肿瘤复发，需要另外两次经蝶手术。在她第三次经蝶窦切除术后，ACTH恢复正常，放射学上没有发现残留肿瘤，患者的皮肤色素沉着改善。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。分娩后一个月，ACTH水平开始上升至5，935 pg/mL的峰值。影像学检查发现两个新的双侧垂体腺瘤，左侧14 mm，右侧7 mm。然后，她开始接受帕瑞肽治疗。治疗两个月后，ACTH降至609 pg/mL，重复垂体MRI显示两个垂体腺瘤的大小间隔性下降，左侧为13 mm，右侧为6 mm。我们报告一位年轻女性，在双侧肾上腺切除术和多次经蝶手术后，多次复发纳尔逊综合征，最终对帕瑞肽有反应。她的情况下，以前没有描述的独特功能是反应帕瑞肽在放疗初治患者，以及快速放射治疗反应。她的病史说明了纳尔逊综合征尚未解决的挑战，并继续需要额外的研究，以确定最佳的管理。
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. 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. 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.