Miyachi Y
First Department of Internal Medicine, Toho University School of Medicine.
Nihon Naibunpi Gakkai Zasshi. 1994 Jan 20;70(1):1-10. doi: 10.1507/endocrine1927.70.1_1.
The separation of Cushing's disease from non-pituitary ACTH-secreting tumors is rarely straightforward. Both a 16-year-old girl and a 56-year-old man exhibited typical clinical features of Cushing's syndrome. In both patients urinary 17OHCS was suppressed by 8mg dexamethasone and plasma cortisol and ACTH responded to intravenous administration of CRH. X-rays of the skull, a computed tomographic scan and a magnetic resonance imaging (MRI) of the sella turcica area showed no significant abnormality. An enhanced MRI of the pituitary gland in the first patient demonstrated a small low-density area which represented microadenoma. In the second patient, an enhanced MRI and inferior sinus sampling failed to demonstrate a pituitary tumor. The patient died of acute myocardial infarction, and an autopsy revealed a bronchial carcinoid in the right upper pulmonary lobe. It is necessary to differentiate an ectopic ACTH-secretion in patients with Cushing's syndrome who show typical biochemical features of Cushing's disease but fail to demonstrate the presence of a pituitary tumor by routine radiological evaluation.
将库欣病与非垂体性促肾上腺皮质激素（ACTH）分泌肿瘤区分开来并非易事。一名16岁女孩和一名56岁男性均表现出库欣综合征的典型临床特征。两名患者的尿17-羟皮质类固醇（17OHCS）均被8毫克地塞米松抑制，血浆皮质醇和促肾上腺皮质激素（ACTH）对静脉注射促肾上腺皮质激素释放激素（CRH）有反应。头颅X线、蝶鞍区计算机断层扫描（CT）和磁共振成像（MRI）均未显示明显异常。首例患者垂体增强MRI显示一个小的低密度区，提示微腺瘤。第二例患者，垂体增强MRI及岩下窦采血均未发现垂体肿瘤。该患者死于急性心肌梗死，尸检发现右上肺叶有支气管类癌。对于表现出库欣病典型生化特征但经常规影像学评估未发现垂体肿瘤的库欣综合征患者，有必要鉴别是否存在异位ACTH分泌。
