An unusual manifestation of olfactory neuroblastoma.
An unusual manifestation of olfactory neuroblastoma.
复制标题
嗅神经母细胞瘤的异常表现。
DOI：
10.1136/bcr-2017-221661
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发表时间：
2018
期刊：
影响因子：
0.9
通讯作者：
Trikudanathan,Subbulaxmi
中科院分区：
文献类型：
--
作者：
Batacchi,Zona;Andeen,NicoleK;Trikudanathan,Subbulaxmi
患者为62岁女性，有11个月鼻出血、嗅觉障碍、鼻塞和间歇性鼻出血症状加重的病史。核磁共振显示上鼻穹隆有一个大肿块。肿块活检显示为嗅神经母细胞瘤。在等待手术期间，她突然出现了严重的近端肌肉无力、嗜睡和腿部浮肿。血糖升高，低血钾性代谢性碱中毒。血清皮质醇水平为95.7µg/dL(8.7~2 2.4µg/dL)，24小时尿皮质醇水平为6962.3µg/24 小时(4.0~5 0.0µg/24 小时)，同时促肾上腺皮质激素水平为171 pg/m L(6-58 pg/m L)，提示皮质醇增多症的来源依赖。随后的大剂量地塞米松抑制试验阳性与异位ACTH产生一致。她接受了右侧鼻部肿块的近全切除，随后进行了放射治疗，皮质醇过多的体征和症状完全消失。
A 62-year-old woman presented with an 11-month history of worsening nasal symptoms of rhinorrhoea, anosmia, nasal congestion and intermittent epistaxis. MRI revealed a large mass in the upper nasal vault. Biopsy of the mass revealed an olfactory neuroblastoma. While waiting resection, she acutely developed severe proximal muscle weakness, lethargy and lower extremity oedema. Blood glucose was elevated, and hypokalaemic metabolic alkalosis was noted. Elevated serum cortisol level of 95.7 µg/dL (8.7–22.4 µg/dL) and markedly elevated 24-hour urinary cortisol level of 6962.3 µg/24 hours (4.0–50.0 µg/24 hours) with concurrent adrenocorticotropic hormone (ACTH) level of 171 pg/mL (6–58 pg/mL) were suggestive of an ACTH-dependent source of hypercortisolism. A subsequent positive high-dose dexamethasone suppression test was consistent with ectopic ACTH production. She underwent near-total resection of the right nasal mass followed by radiotherapy, resulting in complete resolution of signs and symptoms of cortisol excess.