Hashiba Tetsuo, Saitoh Youichi, Asanuma Nobuyuki, Kouhara Haruhiko, Maruo Tomoyuki, Fujinaka Toshiyuki, Kasayama Soji, Yoshimine Toshiki
Department of Neurosurgery, Osaka University Graduate School of Medicine, Japan.
Endocr J. 2006 Apr;53(2):203-8. doi: 10.1507/endocrj.53.203.
Endocrinologic tests sometimes fail to distinguish adrenocorticotropic hormone (ACTH)-secreting pituitary adenoma from ectopic ACTH-secreting tumor. The authors experienced a case of Cushing's disease associated with a pancreatic tumor. Venous sampling contributed to the final diagnosis of Cushing's disease in this complex case, while endocrinologic tests showed paradoxical results. A 54-year-old woman presented with Cushing's syndrome and pancreatic tumor. Magnetic resonance imaging (MRI) failed to reveal a pituitary tumor, but a gadolinium-enhanced tumor with cystic components was seen in the pancreatic tail. Results of conventional endocrinologic tests suggested ectopic ACTH syndrome, but venous sampling including cavernous sinus sampling indicated an ACTH-secreting pituitary adenoma. Transsphenoidal surgery revealed a pituitary microadenoma, and total removal of the tumor was achieved. Postoperative abdominal MRI revealed that the pancreatic tumor diminished gradually without treatment. Selective cavernous sinus sampling was useful for distinguishing ACTH-secreting pituitary adenoma from ectopic ACTH syndrome in this complex case. This was a rare case in which the pancreatic tumor diminished after total removal of the ACTH-secreting pituitary adenoma.
内分泌学检查有时无法区分分泌促肾上腺皮质激素（ACTH）的垂体腺瘤与异位分泌ACTH的肿瘤。作者遇到一例库欣病合并胰腺肿瘤的病例。在这个复杂病例中，静脉采血有助于库欣病的最终诊断，而内分泌学检查结果却相互矛盾。一名54岁女性出现库欣综合征和胰腺肿瘤。磁共振成像（MRI）未发现垂体肿瘤，但在胰尾可见一个有囊性成分的钆增强肿瘤。传统内分泌学检查结果提示异位ACTH综合征，但包括海绵窦采血在内的静脉采血提示为分泌ACTH的垂体腺瘤。经蝶窦手术发现垂体微腺瘤，并实现了肿瘤的完全切除。术后腹部MRI显示胰腺肿瘤未经治疗逐渐缩小。在这个复杂病例中，选择性海绵窦采血有助于区分分泌ACTH的垂体腺瘤与异位ACTH综合征。这是一例罕见的病例，在分泌ACTH的垂体腺瘤完全切除后胰腺肿瘤缩小。
