Morita Hiroyuki, Yasuda Keigo, Nkashima Kazuya, Noritake Nobuyasu, Murayama Masanori, Shimokawa Kuniyasu, Yamakita Noriyoshi, Sano Toshiaki, Sasano Nobuaki, Miura Kiyoshi
Third Department of Internal Medicine, Gifu University School of Medicine, Japan.
Department of Internal Medicine, Inuyama Central Hospital, Japan.
Endocr Pathol. 1994 Jun;5(2):123-130. doi: 10.1007/BF02921380.
A 60-year-old woman with an 8-year history of Cushing's syndrome was evaluated. Biochemical data were consistent with those of Cushing's disease. Plasma ACTH levels responded paradoxically to GnRH. MRI demonstrated a large tumor occupying the sphenoid sinus, which was enhanced by gadolinium diethylenetriamine pentaacetic acid (Gd-DTPA). The pituitary gland was normal in shape and was located in the sella turcica without dislocation. The pituitary gland and the sphenoid tumor could be distinguished by the obvious difference in their MRI intensities. Three consecutive partial resections of the sphenoid tumor were performed, but plasma ACTH and cortisol levels remained high just after the third operation. Histological studies revealed a chromophobe adenoma immunohistochemically positive for ACTH. However, adrenal crisis occurred 3 months after the third operation during reserpine administration ( 1.5 mg/day for approximately 2 mo) for the treatment of Cushing's syndrome due to the residual tumor in the sphenoid sinus. Subsequent MRI showed no change in the tumor shadow, and the paradoxical response of plasma ACTH levels to GnRH remained unchanged. The fourth operation reconfirmed the existence of the ACTH-producing adenoma in the sphenoid sinus. There was no anatomical interaction between the sphenoid tumor and the pituitary gland, and, histologically, no tumor cells were present in the pituitary gland. These findings suggest that the tumor is an ACTH-producing ectopic pituitary adenoma arising from the sphenoid sinus. The patient has been in remission for 4 years on glucocorticoid replacement therapy. The factors responsible for the adrenal crisis were not well understood, although reserpine administration might have had some role.
对一名有8年库欣综合征病史的60岁女性进行了评估。生化数据与库欣病相符。血浆促肾上腺皮质激素（ACTH）水平对促性腺激素释放激素（GnRH）呈反常反应。磁共振成像（MRI）显示一个大肿瘤占据蝶窦，钆喷酸葡胺（Gd-DTPA）增强了肿瘤信号。垂体形态正常，位于蝶鞍内无移位。垂体和蝶窦肿瘤在MRI信号强度上有明显差异，可予以区分。对蝶窦肿瘤进行了连续三次部分切除术，但第三次手术后血浆ACTH和皮质醇水平仍居高不下。组织学研究显示为嫌色性腺瘤，免疫组化ACTH呈阳性。然而，第三次手术后3个月，在使用利血平（1.5mg/天，约2个月）治疗因蝶窦残留肿瘤导致的库欣综合征期间发生了肾上腺危象。随后的MRI显示肿瘤阴影无变化，血浆ACTH水平对GnRH的反常反应也未改变。第四次手术再次证实蝶窦内存在分泌ACTH的腺瘤。蝶窦肿瘤与垂体之间无解剖学上的相互影响，组织学检查显示垂体中无肿瘤细胞。这些发现提示该肿瘤是起源于蝶窦的分泌ACTH的异位垂体腺瘤。该患者接受糖皮质激素替代治疗已缓解4年。尽管利血平的使用可能起了一定作用，但肾上腺危象的相关因素尚不完全清楚。
