He Xin, Spencer-Segal Joanna L
Department of Internal Medicine, Division of Metabolism, Endocrinology & Diabetes, University of Michigan, 1500 East Medical Center Drive, Ann Arbor, MI, 48109, USA.
Michigan Neuroscience Institute, University of Michigan, 205 Zina Pitcher Pl, Ann Arbor, MI, 48109, USA.
Clin Diabetes Endocrinol. 2020 Nov 7;6(1):22. doi: 10.1186/s40842-020-00110-7.
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.
尼尔森综合征是双侧肾上腺切除治疗库欣病后一种广为人知的并发症。对于以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为三联征的尼尔森综合征，目前尚无最佳治疗方案的共识。已对多种药物类别的药物治疗进行了研究。一种已被批准用于治疗库欣病的潜在有前景的药物是帕西瑞肽，它是一种对多种生长抑素受体具有亲和力的生长抑素类似物，包括5型亚型，这是促肾上腺皮质激素肿瘤上表达最高的受体。
一名24岁女性被诊断为库欣病，初始促肾上腺皮质激素水平约为700 - 800 pg/mL。她接受了经蝶窦手术但未缓解，随后进行了双侧肾上腺切除术。在随后的3年里，患者出现皮肤色素沉着、促肾上腺皮质激素反复升高以及肿瘤复发，需要再次进行两次经蝶窦手术。在她第三次经蝶窦切除术后，促肾上腺皮质激素恢复正常，影像学检查未发现残留肿瘤，患者的皮肤色素沉着也有所改善。然后她顺利足月妊娠，在此期间促肾上腺皮质激素水平保持在正常范围内。分娩后一个月，促肾上腺皮质激素水平开始上升，峰值达到5935 pg/mL。影像学检查发现双侧出现两个新的垂体腺瘤，左侧直径14 mm，右侧直径7 mm。随后她开始使用帕西瑞肽治疗。治疗两个月后，促肾上腺皮质激素降至609 pg/mL，垂体MRI复查显示双侧垂体腺瘤大小均有减小，左侧降至13 mm，右侧降至6 mm。
我们报告了一名年轻女性在双侧肾上腺切除和多次经蝶窦手术后尼尔森综合征多次复发的漫长病程，她最终对帕西瑞肽产生了反应。她的病例中以前未描述的独特特征是在未接受过放疗的患者中对帕西瑞肽有反应，以及对治疗的快速影像学反应。她的病史说明了尼尔森综合征尚未解决的挑战以及持续需要更多研究来确定最佳治疗方案。
