Matheny Leslee N, Sarkar Sudipa, Shi Hanyuan, Hu Jiun-Ruey, Harmsen Hannah, Abel Ty W, Jagasia Shubhada M, Bao Shichun
Vanderbilt University Medical Center, Division of Endocrinology, Department of Medicine, Vanderbilt University, 1215 21st Avenue South, Nashville, TN 37232, USA.
Johns Hopkins University School of Medicine, Division of Endocrinology, Diabetes and Metabolism, 5501 Hopkins Bayview Circle, Baltimore, MD 21224, USA.
Case Rep Endocrinol. 2018 Feb 4;2018:6389374. doi: 10.1155/2018/6389374. eCollection 2018.
We present a case of a 52-year-old male who developed Cushing's Syndrome due to ectopic adrenocorticotrophic hormone (ACTH) secretion from a large esthesioneuroblastoma (ENB) of the nasal sinuses. The patient initially presented with polyuria, polydipsia, weakness, and confusion. Computed tomography scan of the head and magnetic resonance imaging showed a 7 cm skull base mass centered in the right cribriform plate without sella involvement. Work-up revealed ACTH-dependent hypercortisolemia, which did not suppress appropriately after high-dose dexamethasone. Subsequent imaging of the chest, abdomen, and pelvis did not reveal other possible ectopic sources of ACTH secretion besides the ENB. His hospital course was complicated by severe hypokalemia and hyperglycemia before successful surgical resection of the tumor, the biopsy of which showed ENB. Postoperatively, his ACTH level dropped below the limit of detection. In the ensuing 4 months, he underwent adjuvant chemoradiation with carboplatin and docetaxel with good response and resolution of hypokalemia and hyperglycemia, with no sign of recurrence as of 30 months postoperatively. His endogenous cortisol production is rising but has not completely recovered.
我们报告一例52岁男性患者，因鼻窦巨大嗅神经母细胞瘤（ENB）异位分泌促肾上腺皮质激素（ACTH）而发生库欣综合征。患者最初表现为多尿、多饮、乏力和意识模糊。头部计算机断层扫描和磁共振成像显示，一个7厘米的颅底肿块，位于右侧筛板，未累及蝶鞍。检查发现ACTH依赖性高皮质醇血症，大剂量地塞米松后不能得到适当抑制。随后对胸部、腹部和骨盆的影像学检查未发现除ENB外其他可能的ACTH异位分泌来源。在成功手术切除肿瘤之前，他的住院过程因严重低钾血症和高血糖而复杂化，肿瘤活检显示为ENB。术后，他的ACTH水平降至检测限以下。在随后的4个月里，他接受了卡铂和多西他赛的辅助放化疗，反应良好，低钾血症和高血糖得到缓解，术后30个月无复发迹象。他的内源性皮质醇分泌正在上升，但尚未完全恢复。
