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首页> 外文期刊>Endocrine journal >Olfactory Neuroblastoma Causing Ectopic ACTH Syndrome
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Olfactory Neuroblastoma Causing Ectopic ACTH Syndrome

机译：引起异位ACTH综合征的嗅觉神经母细胞瘤

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References(27) Cited-By(16) A 39-year-old woman who presented with typical Cushingoid appearance (moon facies, central obesity, purpura) was admitted to our hospital because of pulmonary infection. She was found to have hypertension, severe hypokalemia, and metabolic alkalosis. Endocrine data revealed elevated plasma levels of ACTH and cortisol with lack of circadian rhythm, non-suppressibility to high-dose dexamethasone, and hyperresponsiveness to CRH stimulation. Although no pituitary mass was detected by MRI of the brain, inferior petrosal sinus sampling showed a step-up of central to peripheral ACTH levels; these data are consistent with the diagnosis of Cushing's disease. She was successfully treated with metyrapone to control hypercortisolemia. Ten months later, a mass was detected in the ethmoid sinus, which was surgically removed. After resection of the ethmoid sinus tumor, her Cushingoid features and hypercortisolemia disappeared, but recurred after enlargement of a second mass in the maxillary sinus. After resection of the maxillary sinus tumor, her hypercortisolemia subsided. Histologically, the tumor tissues from both the ethmoid and maxillary sinus were identical and consistent with the diagnosis of olfactory neuroblastoma. Immunohistochemically, the immunoreactivities of ACTH and POMC were positive in the cytoplasm of tumor cells, and immunoreactive ACTH was demonstrated in both tumor tissues. Thus, this is the second rare case with ectopic ACTH syndrome caused by olfactory neuroblastoma thus far reported.
机译：参考文献（27）被引（16）一个39岁，典型的库欣样外观（月相，中心性肥胖，紫癜）的妇女因肺部感染入院。发现她患有高血压，严重的低钾血症和代谢性碱中毒。内分泌数据显示血浆ACTH和皮质醇水平升高，缺乏昼夜节律，对大剂量地塞米松无抑制作用，对CRH刺激反应过度。尽管脑部MRI并未检测到垂体肿块，但下睑窦采样显示中心ACTH水平升高至周围ACTH水平。这些数据与库欣氏病的诊断是一致的。她已成功接受甲吡酮治疗以控制高皮质醇血症。十个月后，在筛窦中发现了肿块，并通过手术将其切除。筛窦窦肿瘤切除后，她的Cushingoid特征和高皮质醇血症消失，但在上颌窦内第二个肿块增大后复发。上颌窦肿瘤切除后，她的高皮质醇血症消退。组织学上，筛骨和上颌窦的肿瘤组织相同，与嗅觉神经母细胞瘤的诊断一致。在免疫组织化学上，ACTH和POMC的免疫反应在肿瘤细胞的细胞质中是阳性的，并且在两种肿瘤组织中都显示出免疫反应性ACTH。因此，这是迄今为止由嗅觉神经母细胞瘤引起的第二例罕见的异位ACTH综合征。

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